Disease on EC 5.6.1.6 - channel-conductance-controlling ATPase
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Abscess
CFTR Depletion Confers Hypersusceptibility to Mycobacterium fortuitum in a Zebrafish Model.
Acute Kidney Injury
Prerenal azotemia from excessive sweating in an adult with a cystic fibrosis gene mutation.
Acute Lung Injury
Association of cystic fibrosis transmembrane conductance regulator gene variants with acute lung injury in African American children with pneumonia.
Acute Lung Injury
Cystic fibrosis transmembrane conductance regulator ameliorates lipopolysaccharide-induced acute lung injury by inhibiting autophagy through PI3K/AKT/mTOR pathway in mice.
Acute Lung Injury
Cystic fibrosis transmembrane conductance regulator modulates acute lung injury: evidence from a genetic association study*.
Acute Lung Injury
Novel role for cystic fibrosis transmembrane conductance regulator in alveolar fluid clearance in lipopolysaccharide-induced acute lung injury in mice.
Adenocarcinoma
Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer.
Adenocarcinoma
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations and risk for pancreatic adenocarcinoma.
Adenocarcinoma
Cystic fibrosis transmembrane regulator gene carrier status is a risk factor for young onset pancreatic adenocarcinoma.
Adenocarcinoma
Delta F508 CFTR localizes in the endoplasmic reticulum-Golgi intermediate compartment in cystic fibrosis cells.
Adenocarcinoma
MUC4 expression is regulated by cystic fibrosis transmembrane conductance regulator in pancreatic adenocarcinoma cells via transcriptional and post-translational mechanisms.
Adenocarcinoma
Nicotine Induces Progressive Properties of Lung Adenocarcinoma A549 Cells by Inhibiting Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Expression and Plasma Membrane Localization.
Adenocarcinoma
Subject Review: Pancreatic Ductal Adenocarcinoma in the Setting of Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator Gene: Case Report and Review of the Literature.
Adenocarcinoma of Lung
CFTR in Calu-3 human airway cells: channel properties and role in cAMP-activated Cl- conductance.
Adenocarcinoma of Lung
Nicotine Induces Progressive Properties of Lung Adenocarcinoma A549 Cells by Inhibiting Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Expression and Plasma Membrane Localization.
Adenocarcinoma of Lung
[Impact of Cystic Fibrosis Transmembrane Conductance Regulator on Malignant? Properties of KRAS Mutant Lung Adenocarcinoma A549 Cells].
Adenoma
Loss of downregulated in adenoma (DRA) impairs mucosal HCO(3) (-) secretion in murine ileocolonic inflammation.
Adenoma
Lubiprostone Targets Prostanoid Signaling and Promotes Ion Transporter Trafficking, Mucus Exocytosis, and Contractility.
Adrenoleukodystrophy
[PMP70, the 70-kDa peroxisomal membrane protein: a member of the ATP-binding cassette transporters]
Airway Obstruction
Assisting PNA transport through cystic fibrosis human airway epithelia with biodegradable hybrid lipid-polymer nanoparticles.
Airway Obstruction
Cystic Fibrosis Transmembrane Conductance Regulator Function in Airway Smooth Muscle. A Novel Role in Cystic Fibrosis Airway Obstruction.
Airway Obstruction
Neuraminidase inhibitors for the treatment of influenza infection in people with cystic fibrosis.
Alkalosis
A specific cystic fibrosis mutation (T3381) associated with the phenotype of isolated hypotonic dehydration.
Alzheimer Disease
Reduced expression of the cystic fibrosis transmembrane conductance regulator gene in the hypothalamus of patients with Alzheimer's disease.
Arthritis, Rheumatoid
Increased frequency of cystic fibrosis deltaF508 mutation in bronchiectasis associated with rheumatoid arthritis.
Aspergillosis, Allergic Bronchopulmonary
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in allergic bronchopulmonary aspergillosis.
Aspergillosis, Allergic Bronchopulmonary
Frequency of cystic fibrosis transmembrane conductance regulator gene mutations and 5T allele in patients with allergic bronchopulmonary aspergillosis.
Aspergillosis, Allergic Bronchopulmonary
Link between CFTR mutations and ABPA: a systematic review and meta-analysis.
Asthma
Association between Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations and Susceptibility for Childhood Asthma in Korea.
Asthma
Association of S549N and IVS8-5T Splice Variants with Bronchial Asthma and Its Severity in Indian Children.
Asthma
Asthma and pulmonary function abnormalities in heterozygotes for cystic fibrosis transmembrane regulator gene mutations.
Asthma
CFTR gene mutations and asthma in the Norwegian Environment and Childhood Asthma study.
Asthma
CFTR gene mutations--including three novel nucleotide substitutions--and haplotype background in patients with asthma, disseminated bronchiectasis and chronic obstructive pulmonary disease.
Asthma
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Asians with chronic pulmonary disease: a pilot study.
Asthma
Cystic fibrosis transmembrane conductance regulator gene abnormalities in patients with asthma and recurrent neutrophilic bronchitis.
Asthma
Genetic and biochemical markers of obstructive lung disease in the general population.
Asthma
Role of the cystic fibrosis transmembrane conductance channel in human airway smooth muscle.
Autoimmune Pancreatitis
Cystic fibrosis transmembrane conductance regulator gene variants are associated with autoimmune pancreatitis and slow response to steroid treatment.
Azoospermia
A novel hemizygous loss-of-function mutation in ADGRG2 causes male infertility with congenital bilateral absence of the vas deferens.
Azoospermia
A novel mutation (-195C>A) in the promoter region of CFTR gene is associated with Chinese Congenital Bilateral Absence of Vas Deferens (CBAVD).
Azoospermia
A Survey of the Common Mutations and IVS8-Tn Polymorphism of Cystic Fibrosis Transmembrane Conductance Regulator Gene in Infertile Men with Nonobstructive Azoospermia and CBAVD in Iranian Population
Azoospermia
Analysis of cystic fibrosis transmembrane regulator and azoospermia factor polymorphisms in infertile men in relation to other abnormalities.
Azoospermia
Andrological findings in infertile men with two (biallelic) CFTR mutations: results of a multicentre study in Germany and Austria comprising 71 patients.
Azoospermia
Birth after intracytoplasmic injection of epididymal sperm from a man with congenital bilateral absence of the vas deferens who had a robertsonian translocation.
Azoospermia
Cystic fibrosis transmembrane conductance regulator and obstructive azoospermia.
Azoospermia
Cytogenetic analysis of azoospermic patients: karyotype comparison of peripheral blood lymphocytes and testicular tissue.
Azoospermia
Different cystic fibrosis transmembrane conductance regulator mutations in chinese men with congenital bilateral absence of vas deferens and other acquired obstructive azoospermia.
Azoospermia
Genetic markers of male infertility: Y chromosome microdeletions and cystic fibrosis transmembrane conductance gene mutations.
Azoospermia
Improved detection of cystic fibrosis mutations in infertility patients with DNA sequence analysis.
Azoospermia
Novel cause of hereditary obstructive azoospermia: a T2 allele in the CFTR gene.
Azoospermia
Prevalence of CBAVD in azoospermic men carrying pathogenic CFTR mutations - Evaluated in a cohort of 639 non-vasectomized azoospermic men.
Azoospermia
Proportion of cystic fibrosis gene mutations not detected by routine testing in men with obstructive azoospermia.
Azoospermia
Re: Lu et al.: Different cystic fibrosis transmembrane conductance regulator mutations in Chinese men with congenital bilateral absence of the vas deferens and other acquired obstructive azoospermia (Urology 2013;82:824-828).
Azoospermia
Screening of ?F508 mutation and IVS8-poly T polymorphism in CFTR gene in Tunisian infertile men without CBAVD.
Azoospermia
Seminal plasma characteristics as indicators of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in men with obstructive azoospermia.
Azoospermia
The CFTR polymorphisms poly-T, TG-repeats and M470V in Chinese males with congenital bilateral absence of the vas deferens.
Azoospermia
Transmission of male infertility and intracytoplasmic sperm injection (mini-review).
Azoospermia
Truncating Mutations in the Adhesion G Protein-Coupled Receptor G2 Gene ADGRG2 Cause an X-Linked Congenital Bilateral Absence of Vas Deferens.
Azoospermia
Variants in CFTR untranslated regions are associated with congenital bilateral absence of the vas deferens.
Bacterial Infections
Counteracting suppression of CFTR and voltage-gated K+ channels by a bacterial pathogenic factor with the natural product tannic acid.
Bacterial Infections
Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.
Bacterial Infections
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function.
Bacterial Infections
Dysfunctional CFTR alters the bactericidal activity of human macrophages against Pseudomonas aeruginosa.
Bacterial Infections
Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice.
Bacterial Infections
IL-17 primes airway epithelial cells lacking functional Cystic Fibrosis Transmembrane conductance Regulator (CFTR) to increase NOD1 responses.
Bacterial Infections
Inflammation in cystic fibrosis airways: relationship to increased bacterial adherence.
Bacterial Infections
Loss of cystic fibrosis transmembrane conductance regulator function enhances activation of p38 and ERK MAPKs, increasing interleukin-6 synthesis in airway epithelial cells exposed to Pseudomonas aeruginosa.
Bacterial Infections
Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis.
Bacterial Infections
Progress toward generating a ferret model of cystic fibrosis by somatic cell nuclear transfer.
Bacterial Infections
Resveratrol and ivacaftor are additive G551D CFTR-channel potentiators: therapeutic implications for cystic fibrosis sinus disease.
Bacterial Infections
Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice.
Bacterial Infections
The Application of Bicarbonate Recovers the Chemical-Physical Properties of Airway Surface Liquid in Cystic Fibrosis Epithelia Models.
Bacterial Infections
Tyrosine kinase c-Src constitutes a bridge between cystic fibrosis transmembrane regulator channel failure and MUC1 overexpression in cystic fibrosis.
Bacterial Infections
Unravelling the Regions of Mutant F508del-CFTR More Susceptible to the Action of Four Cystic Fibrosis Correctors.
Basal Cell Nevus Syndrome
Visualization of oligonucleotide probes and point mutations in interphase nuclei and DNA fibers using rolling circle DNA amplification.
Bone Diseases
Bone disease in cystic fibrosis: new pathogenic insights opening novel therapies.
Bone Diseases
Cystic fibrosis and bone disease: defective osteoblast maturation with the f508del mutation in cystic fibrosis transmembrane conductance regulator.
Bone Diseases
Persistent osteopenia in adult cystic fibrosis transmembrane conductance regulator-deficient mice.
Bone Diseases
The F508del mutation in cystic fibrosis transmembrane conductance regulator gene impacts bone formation.
Bone Diseases, Metabolic
CFTR-deficient pigs display alterations of bone microarchitecture and composition at birth.
Breast Neoplasms
Promoter hypermethylation of the CFTR gene as a novel diagnostic and prognostic marker of breast cancer.
Breast Neoplasms
[MDR (multidrug resistance) in hepatocarcinoma clinical-therapeutic implications]
Bronchiectasis
Analysis of CFTR Gene Variants in Idiopathic Bronchiectasis in Serbian Children.
Bronchiectasis
Assessing effects of inhaled antibiotics in adults with non-cystic fibrosis bronchiectasis--experiences from recent clinical trials.
Bronchiectasis
CFTR gene mutations--including three novel nucleotide substitutions--and haplotype background in patients with asthma, disseminated bronchiectasis and chronic obstructive pulmonary disease.
Bronchiectasis
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
Bronchiectasis
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Asians with chronic pulmonary disease: a pilot study.
Bronchiectasis
Cystic Fibrosis Transmembrane Conductance Regulator Channel Dysfunction in Non-cystic Fibrosis Bronchiectasis.
Bronchiectasis
Increased frequency of cystic fibrosis deltaF508 mutation in bronchiectasis associated with rheumatoid arthritis.
Bronchiectasis
Multi-dimensional clinical phenotyping of a national cohort of adult cystic fibrosis patients.
Bronchiectasis
Novel cftr gene sequence variation in serbian patient with idiopathic disseminated bronchiectasis.
Bronchiectasis
Progression of early structural lung disease in young children with cystic fibrosis assessed using CT.
Bronchiectasis
Prospective analysis of cystic fibrosis transmembrane regulator mutations in adults with bronchiectasis or pulmonary nontuberculous mycobacterial infection.
Bronchiectasis
Quantification of CFTR splice variants in adults with disseminated bronchiectasis, using the TaqMan fluorogenic detection system.
Bronchiectasis
Role of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in patients with chronic sinopulmonary disease.
Bronchiectasis
Sequence analysis of the cystic fibrosis gene in patients with disseminated bronchiectatic lung disease. Application in the identification of a cystic fibrosis patient with atypical clinical course.
Bronchitis
Cystic fibrosis transmembrane conductance regulator gene abnormalities in patients with asthma and recurrent neutrophilic bronchitis.
Bronchitis, Chronic
Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis and Other Diseases of Mucus Clearance.
Bronchitis, Chronic
Cigarette Smoke-Induced Acquired Dysfunction of Cystic Fibrosis Transmembrane Conductance Regulator in the Pathogenesis of Chronic Obstructive Pulmonary Disease.
Bronchitis, Chronic
Cigarette Smoke-induced Ca2+ Release Leads to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Dysfunction.
Bronchitis, Chronic
Common CFTR mutations are not likely to predispose to chronic bronchitis in northern Germany.
Bronchitis, Chronic
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
Bronchitis, Chronic
Cystic fibrosis transmembrane conductance regulator activation by roflumilast contributes to therapeutic benefit in chronic bronchitis.
Bronchitis, Chronic
Detection of CFTR gene mutations in patients suffering from chronic bronchitis.
Bronchitis, Chronic
Hydrator Therapies for Chronic Bronchitis. Lessons from Cystic Fibrosis.
Bronchitis, Chronic
Roflumilast reverses CFTR-mediated ion transport dysfunction in cigarette smoke-exposed mice.
Bronchitis, Chronic
TGF-beta 1 and Cigarette Smoke Inhibit the Ability of ?2-agonists to Enhance Epithelial Permeability.
Bronchitis, Chronic
Therapeutic Approaches to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis.
Carcinogenesis
A novel role and mechanism of cystic fibrosis transmembrane conductance regulator in bisphenol A-induced prostate cancer.
Carcinogenesis
The promoter of the human cystic fibrosis transmembrane conductance regulator gene directing SV40 T antigen expression induces malignant proliferation of ependymal cells in transgenic mice.
Carcinoma
Bilateral carcinoma in situ of the testis and cystic fibrosis transmembrane conductance regulator (CFTR) mutation in an azoospermic patient with late-onset 21beta-hydroxylase deficiency.
Carcinoma
CFTR Functions as a Tumor Suppressor and Is Regulated by DNA Methylation in Colorectal Cancer.
Carcinoma
Characterization of the cAMP response element of the cystic fibrosis transmembrane conductance regulator gene promoter.
Carcinoma
Cystic fibrosis transmembrane conductance regulator is helpful in the distinction of extra-mammary Paget's disease from squamous cell carcinoma in situ (Bowen's disease).
Carcinoma
Effects of mutations in cAMP-dependent protein kinase on chloride efflux in Caco-2 human colonic carcinoma cells.
Carcinoma
Induction of expression of the cystic fibrosis transmembrane conductance regulator.
Carcinoma
NF-kappaB activation is involved in regulation of cystic fibrosis transmembrane conductance regulator (CFTR) by interleukin-1beta.
Carcinoma
Swelling-activated, cystic fibrosis transmembrane conductance regulator-augmented ATP release and Cl- conductances in murine C127 cells.
Carcinoma in Situ
Bilateral carcinoma in situ of the testis and cystic fibrosis transmembrane conductance regulator (CFTR) mutation in an azoospermic patient with late-onset 21beta-hydroxylase deficiency.
Carcinoma in Situ
Cystic fibrosis transmembrane conductance regulator is helpful in the distinction of extra-mammary Paget's disease from squamous cell carcinoma in situ (Bowen's disease).
Carcinoma, Non-Small-Cell Lung
The cystic fibrosis transmembrane conductance regulator as a biomarker in non-small cell lung cancer.
Carcinoma, Squamous Cell
Cystic fibrosis transmembrane conductance regulator is helpful in the distinction of extra-mammary Paget's disease from squamous cell carcinoma in situ (Bowen's disease).
Cardiomyopathies
Heterozygous Cystic Fibrosis Transmembrane Regulator Gene Missense Variants Are Associated With Worse Cardiac Function in Patients With Duchenne Muscular Dystrophy.
Cardiomyopathies
Technological advances shed light on left ventricular cardiac disturbances in cystic fibrosis.
Celiac Disease
A pathogenic role for cystic fibrosis transmembrane conductance regulator in celiac disease.
Celiac Disease
Cystic fibrosis transmembrane conductance regulator (CFTR) and autophagy: hereditary defects in cystic fibrosis versus gluten-mediated inhibition in celiac disease.
channel-conductance-controlling atpase deficiency
Acquired Cystic Fibrosis Transmembrane Conductance Regulator Deficiency.
channel-conductance-controlling atpase deficiency
Clinical practice and genetic counseling for cystic fibrosis and CFTR-related disorders.
channel-conductance-controlling atpase deficiency
Cystic fibrosis transmembrane conductance regulator deficiency exacerbates islet cell dysfunction after beta-cell injury.
channel-conductance-controlling atpase deficiency
Defects in Gallbladder Emptying and Bile Acid Homeostasis in Mice with Cystic Fibrosis Transmembrane Conductance Regulator Deficiencies.
channel-conductance-controlling atpase deficiency
Diet-Induced Dysbiosis and Genetic Background Synergize With Cystic Fibrosis Transmembrane Conductance Regulator Deficiency to Promote Cholangiopathy in Mice.
channel-conductance-controlling atpase deficiency
Self-assembled peptide-poloxamine nanoparticles enable in vitro and in vivo genome restoration for cystic fibrosis.
channel-conductance-controlling atpase deficiency
Transcriptional adaptation to cystic fibrosis transmembrane conductance regulator deficiency.
Cholangitis, Sclerosing
CFTR dysfunction predisposes to fibrotic liver disease in a murine model.
Cholangitis, Sclerosing
Cystic fibrosis transmembrane conductance regulator (CFTR) gene defects in patients with primary sclerosing cholangitis.
Cholangitis, Sclerosing
Cystic fibrosis transmembrane conductance regulator gene polymorphisms in patients with primary sclerosing cholangitis.
Cholangitis, Sclerosing
Cytokine-stimulated nitric oxide production inhibits adenylyl cyclase and cAMP-dependent secretion in cholangiocytes.
Cholangitis, Sclerosing
Decreased Peroxisome Proliferator Activated Receptor alpha Is Associated with Bile Duct Injury in Cystic Fibrosis Transmembrane Conductance Regulator-/- Mice.
Cholangitis, Sclerosing
Lack of association of common cystic fibrosis transmembrane conductance regulator gene mutations with primary sclerosing cholangitis.
Cholangitis, Sclerosing
Novel association of HLA-haplotypes with primary sclerosing cholangitis (PSC) in a southern European population.
Cholangitis, Sclerosing
Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases.
Cholangitis, Sclerosing
Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function.
Cholangitis, Sclerosing
Survey of cystic fibrosis transmembrane conductance regulator genotypes in primary sclerosing cholangitis.
Cholera
AC6 is the major adenylate cyclase forming a diarrheagenic protein complex with cystic fibrosis transmembrane conductance regulator in cholera.
Cholera
Activation of AMP-activated protein kinase by a plant-derived dihydroisosteviol in human intestinal epithelial cell.
Cholera
Identification of new small molecule inhibitors of cystic fibrosis transmembrane conductance regulator protein: in vitro and in vivo studies.
Cholera
Lectin conjugates as potent, nonabsorbable CFTR inhibitors for reducing intestinal fluid secretion in cholera.
Cholera
Luminally active, nonabsorbable CFTR inhibitors as potential therapy to reduce intestinal fluid loss in cholera.
Cholera
Lysophosphatidic acid inhibits cholera toxin-induced secretory diarrhea through CFTR-dependent protein interactions.
Cholera
Mutual Enhancement of Virulence by Enterotoxigenic and Enteropathogenic Escherichia coli.
Cholera
Nanomolar CFTR inhibition by pore-occluding divalent polyethylene glycol-malonic acid hydrazides.
Cholera
Novel action of the chalcone isoliquiritigenin as a cystic fibrosis transmembrane conductance regulator (CFTR) inhibitor: potential therapy for cholera and polycystic kidney disease.
Cholera
Novel Action of the Chalcone Isoliquiritigenin as a Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Inhibitor: Potential Therapy for Cholera and Polycystic Kidney Disease.
Cholera
Thiazolidinone CFTR inhibitors with improved water solubility identified by structure-activity analysis.
Cholestasis
Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors.
Cholestasis
Manifestation of cystic fibrosis transmembrane regulator (CFTR) in hepatic ductal structures and renal tubules of female rats with experimental cholestasis of pregnancy.
Ciliary Motility Disorders
Frequency of Cystic Fibrosis Transmembrane Conductance Regulator Variants in Individuals Evaluated for Primary Ciliary Dyskinesia.
Coinfection
Unique microbial communities persist in individual cystic fibrosis patients throughout a clinical exacerbation.
Colitis
Expression of voltage-gated potassium channels in human and mouse colonic carcinoma.
Colitis
Lactobacillus casei protects dextran sodium sulfate- or rapamycin-induced colonic inflammation in the mouse.
Colitis, Ulcerative
CFTR dysfunction predisposes to fibrotic liver disease in a murine model.
Colitis, Ulcerative
Upregulation of CFTR expression but not SLC26A3 and SLC9A3 in ulcerative colitis.
Colonic Neoplasms
Activation of the cystic fibrosis transmembrane conductance regulator by cGMP in the human colonic cancer cell line, Caco-2.
Congenital Abnormalities
Nonvisualization of the Fetal Gallbladder: Can Levels of Gamma-Glutamyl Transpeptidase in Amniotic Fluid Predict Fetal Prognosis?
Cough
Assessing effects of inhaled antibiotics in adults with non-cystic fibrosis bronchiectasis--experiences from recent clinical trials.
Cough
Nutritional status and eating disorders: neglected risks factor for nontuberculous mycobacterial lung disease?
Cough
Unique mutations of the cystic fibrosis transmembrane conductance regulator gene of three cases of cystic fibrosis in Nagasaki, Japan.
COVID-19
A Scientific Rationale for Using Cystic Fibrosis Transmembrane Conductance Regulator Therapeutics in COVID-19 Patients.
Creutzfeldt-Jakob Syndrome
Gene SNPs and mutations in clinical genetic testing: haplotype-based testing and analysis.
Cryptorchidism
Cryptorchidism-induced CFTR down-regulation results in disruption of testicular tight junctions through up-regulation of NF-?B/COX-2/PGE2.
Cystic Fibrosis
"Reactance inversion" at low frequencies in a child undergoing treatment of a cystic fibrosis exacerbation.
Cystic Fibrosis
"Sero-switch" adenovirus-mediated in vivo gene transfer: circumvention of anti-adenovirus humoral immune defenses against repeat adenovirus vector administration by changing the adenovirus serotype.
Cystic Fibrosis
"Summer hypokalemia" as an initial presentation of cystic fibrosis in a morbidly obese African American adult: case report.
Cystic Fibrosis
'CFTR-opathies': disease phenotypes associated with cystic fibrosis transmembrane regulator gene mutations.
Cystic Fibrosis
1-BENZYLSPIRO[PIPERIDINE-4,1'-PYRIDO[3,4-b]indole] 'co-potentiators' for minimal function CFTR mutants.
Cystic Fibrosis
150 mM HCO3(-)--how does the pancreas do it? Clues from computer modelling of the duct cell.
Cystic Fibrosis
16alpha-Bromoepiandrosterone (HE2000) limits non-productive inflammation and stimulates immunity in lungs.
Cystic Fibrosis
17beta-estradiol potentiates the cardiac cystic fibrosis transmembrane conductance regulator chloride current in guinea-pig ventricular myocytes.
Cystic Fibrosis
2,3-butanedione monoxime affects cystic fibrosis transmembrane conductance regulator channel function through phosphorylation-dependent and phosphorylation-independent mechanisms: the role of bilayer material properties.
Cystic Fibrosis
2-(dialkylamino)-4H-1-benzopyran-4-one derivatives modify chloride conductance in CFTR expressing cells.
Cystic Fibrosis
3-(2-Benzyloxyphenyl)isoxazoles and isoxazolines: synthesis and evaluation as CFTR activators.
Cystic Fibrosis
40 kilobase deletion (CF 40 kb del 4-10) removes exons 4 to 10 of the Cystic Fibrosis Transmembrane Conductance Regulator gene.
Cystic Fibrosis
5'-adenosine monophosphate mediated cooling treatment enhances ?F508-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) stability in vivo.
Cystic Fibrosis
5'-Adenylylimidodiphosphate does not activate CFTR chloride channels in cell-free patches of membrane.
Cystic Fibrosis
5-Hydroxytryptamine contributes significantly to a reflex pathway by which the duodenal mucosa protects itself from gastric acid injury.
Cystic Fibrosis
8-cyclopentyl-1,3-dipropylxanthine and other xanthines differentially bind to the wild-type and delta F508 first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
8-iso-PGE2 stimulates anion efflux from airway epithelial cells via the EP4 prostanoid receptor.
Cystic Fibrosis
? potential changing nanoparticles as cystic fibrosis transmembrane conductance regulator gene delivery system: an in vitro evaluation.
Cystic Fibrosis
?-eudesmol but not atractylodin exerts an inhibitory effect on CFTR-mediated chloride transport in human intestinal epithelial cells.
Cystic Fibrosis
?-Fetoprotein Gene Delivery to the Nasal Epithelium of Nonhuman Primates by Human Parainfluenza Viral Vectors.
Cystic Fibrosis
?1 Syntrophin Supports Autophagy Initiation and Protects against Cerulein-Induced Acute Pancreatitis.
Cystic Fibrosis
?2 adrenoceptor signaling regulates ion transport in 16HBE14o- human airway epithelial cells.
Cystic Fibrosis
?2-Adrenoreceptor Agonist Inhalation During Ex Vivo Lung Perfusion Attenuates Lung Injury.
Cystic Fibrosis
???-ENaC is inhibited by CFTR but stimulated by cAMP in Xenopus laevis oocytes.
Cystic Fibrosis
?F508 CFTR surface stability is regulated by DAB2 and CHIP-mediated ubiquitination in post-endocytic compartments.
Cystic Fibrosis
?F508-CFTR correctors: synthesis and evaluation of thiazole-tethered imidazolones, oxazoles, oxadiazoles, and thiadiazoles.
Cystic Fibrosis
?F508-CFTR Modulator Screen Based on Cell Surface Targeting of a Chimeric Nucleotide Binding Domain 1 Reporter.
Cystic Fibrosis
A 32-bp deletion (2991del32) in the cystic fibrosis gene associated with CFTR mRNA reduction.
Cystic Fibrosis
A 96-well formatted method for exon and exon/intron boundary full sequencing of the CFTR gene.
Cystic Fibrosis
A BAC Transgene Expressing Human CFTR under Control of Its Regulatory Elements Rescues Cftr Knockout Mice.
Cystic Fibrosis
A balance between activating and repressive histone modifications regulates cystic fibrosis transmembrane conductance regulator (CFTR) expression in vivo.
Cystic Fibrosis
A Bifidobacterium probiotic strain and its soluble factors alleviate chloride secretion by human intestinal epithelial cells.
Cystic Fibrosis
A bird eye view on cystic fibrosis: An underestimated multifaceted chronic disorder.
Cystic Fibrosis
A C-terminal motif found in the beta2-adrenergic receptor, P2Y1 receptor and cystic fibrosis transmembrane conductance regulator determines binding to the Na+/H+ exchanger regulatory factor family of PDZ proteins.
Cystic Fibrosis
A case report of CUAVD with azoospermia: a proposal of a rational diagnostic approach.
Cystic Fibrosis
A Cdc48p-associated factor modulates endoplasmic reticulum-associated degradation, cell stress, and ubiquitinated protein homeostasis.
Cystic Fibrosis
A CFTR chloride channel activator prevents HrpN(ea)-induced cell death in Arabidopsis thaliana suspension cells.
Cystic Fibrosis
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.
Cystic Fibrosis
A chimeric type 2 adenovirus vector with a type 17 fiber enhances gene transfer to human airway epithelia.
Cystic Fibrosis
A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis.
Cystic Fibrosis
A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein.
Cystic Fibrosis
A cluster of highly polymorphic dinucleotide repeats in intron 17b of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
A cluster of negative charges at the amino terminal tail of CFTR regulates ATP-dependent channel gating.
Cystic Fibrosis
A combined analysis of the cystic fibrosis transmembrane conductance regulator: implications for structure and disease models.
Cystic Fibrosis
A common mechanism for cystic fibrosis transmembrane conductance regulator protein activation by genistein and benzimidazolone analogs.
Cystic Fibrosis
A comparative genomic analysis of the cow, pig, and human CFTR genes identifies potential intronic regulatory elements.
Cystic Fibrosis
A comparison of 14 antibodies for the biochemical detection of the cystic fibrosis transmembrane conductance regulator protein.
Cystic Fibrosis
A comparison of high-resolution melting analysis with denaturing high-performance liquid chromatography for mutation scanning: cystic fibrosis transmembrane conductance regulator gene as a model.
Cystic Fibrosis
A comparison of the performance of voltammetric aptasensors for glycated haemoglobin on different carbon nanomaterials-modified screen printed electrodes.
Cystic Fibrosis
A complex intronic enhancer regulates expression of the CFTR gene by direct interaction with the promoter.
Cystic Fibrosis
A comprehensive analysis of aquaporin and secretory related gene expression in neonate and adult cholangiocytes.
Cystic Fibrosis
A conditional probability analysis of cystic fibrosis transmembrane conductance regulator gating indicates that ATP has multiple effects during the gating cycle.
Cystic Fibrosis
A conserved region of the R domain of cystic fibrosis transmembrane conductance regulator is important in processing and function.
Cystic Fibrosis
A controlled study of adenoviral-vector-mediated gene transfer in the nasal epithelium of patients with cystic fibrosis.
Cystic Fibrosis
A cross-species analysis of the cystic fibrosis transmembrane conductance regulator. Potential functional domains and regulatory sites.
Cystic Fibrosis
A current review of the safety of cystic fibrosis transmembrane conductance regulator modulators.
Cystic Fibrosis
A cyclic nucleotide PDE5 inhibitor corrects defective mucin secretion in submandibular cells containing antibody directed against the cystic fibrosis transmembrane conductance regulator protein.
Cystic Fibrosis
A cystic fibrosis allele encoding missense mutations in both nucleotide binding folds of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
A cystic fibrosis bronchial epithelial cell line: immortalization by adeno-12-SV40 infection.
Cystic Fibrosis
A cystic fibrosis transmembrane conductance regulator splice variant with partial penetrance associated with variable cystic fibrosis presentations.
Cystic Fibrosis
A deletion mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) locus: Delta I507.
Cystic Fibrosis
A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo.
Cystic Fibrosis
A divergent CFTR homologue: highly regulated salt transport in the euryhaline teleost F. heteroclitus.
Cystic Fibrosis
A domain mimic increases DeltaF508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia.
Cystic Fibrosis
A finger sweat chloride test for the detection of a high-risk group of chronic pancreatitis.
Cystic Fibrosis
A functional CFTR assay using primary cystic fibrosis intestinal organoids.
Cystic Fibrosis
A functional CFTR protein is required for mouse intestinal cAMP-, cGMP- and Ca(2+)-dependent HCO3- secretion.
Cystic Fibrosis
A functional R domain from cystic fibrosis transmembrane conductance regulator is predominantly unstructured in solution.
Cystic Fibrosis
A Genome-Wide Analysis of Open Chromatin in Human Epididymis Epithelial Cells Reveals Candidate Regulatory Elements for Genes Coordinating Epididymal Function.
Cystic Fibrosis
A Genotypic-Oriented View of CFTR Genetics Highlights Specific Mutational Patterns Underlying Clinical Macrocategories of Cystic Fibrosis.
Cystic Fibrosis
A Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expression.
Cystic Fibrosis
A haplotype-based molecular analysis of CFTR mutations associated with respiratory and pancreatic diseases.
Cystic Fibrosis
A heteromeric complex of the two nucleotide binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) mediates ATPase activity.
Cystic Fibrosis
A hierarchy of signals regulates entry of membrane proteins into the ciliary membrane domain in epithelial cells.
Cystic Fibrosis
A High Level of Soluble CD40L Is Associated with P. aeruginosa Infection in Patients with Cystic Fibrosis.
Cystic Fibrosis
A high prevalence of genetic polymorphisms in idiopathic and alcohol-associated chronic pancreatitis patients in Ireland.
Cystic Fibrosis
A Homogeneous Cell-Based Halide-Sensitive Yellow Fluorescence Protein Assay to Identify Modulators of the Cystic Fibrosis Transmembrane Conductance Regulator Ion Channel.
Cystic Fibrosis
A human ciliopathy reveals essential functions for NEK10 in airway mucociliary clearance.
Cystic Fibrosis
A kinase-regulated mechanism controls CFTR channel gating by disrupting bivalent PDZ domain interactions.
Cystic Fibrosis
A large deletion causes apparent homozygosity for the D1152H mutation in the cystic fibrosis transmembrane regulator (CFTR) gene.
Cystic Fibrosis
A Link Between a Common Mutation in CFTR and Impaired Innate and Adaptive Viral Defense.
Cystic Fibrosis
A macromolecular complex of beta 2 adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phosphoprotein 50 is regulated by PKA.
Cystic Fibrosis
A method for the amplification of unknown flanking DNA: targeted inverted repeat amplification.
Cystic Fibrosis
A microRNA network regulates expression and biosynthesis of wild-type and {Delta}F508 mutant cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
A missense cystic fibrosis transmembrane conductance regulator mutation with variable phenotype.
Cystic Fibrosis
A missense mutation (F87L) in exon 3 of the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
A missense mutation in SLC26A3 is associated with human male subfertility and impaired activation of CFTR.
Cystic Fibrosis
A model-based economic evaluation of four newborn screening strategies for cystic fibrosis in Flanders, Belgium.
Cystic Fibrosis
A Modified Alderman-Grant Coil makes possible an efficient cross-coil probe for high field solid-state NMR of lossy biological samples.
Cystic Fibrosis
A molecular mechanism for aberrant CFTR-dependent HCO(3)(-) transport in cystic fibrosis.
Cystic Fibrosis
A monoclonal antibody prevents aggregation of the NBD1 domain of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
A monomer is the minimum functional unit required for channel and ATPase activity of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
A murine tracheal culture system to investigate parameters affecting gene therapy for cystic fibrosis.
Cystic Fibrosis
A mutation in CFTR modifies the effects of the adenylate kinase inhibitor Ap5A on channel gating.
Cystic Fibrosis
A mutation in the cystic fibrosis transmembrane conductance regulator gene associated with elevated sweat chloride concentrations in the absence of cystic fibrosis.
Cystic Fibrosis
A mutation in the cystic fibrosis transmembrane conductance regulator generates a novel internalization sequence and enhances endocytic rates.
Cystic Fibrosis
A mutation of the epithelial sodium channel associated with atypical cystic fibrosis increases channel open probability and reduces Na+ self inhibition.
Cystic Fibrosis
A natural plant-derived dihydroisosteviol prevents cholera toxin-induced intestinal fluid secretion.
Cystic Fibrosis
A naturally occurring sequence variation that creates a YY1 element is associated with increased cystic fibrosis transmembrane conductance regulator gene expression.
Cystic Fibrosis
A neutral variant involved in a complex CFTR allele contributes to a severe cystic fibrosis phenotype.
Cystic Fibrosis
A Neutralizing Aptamer to TGFBR2 and miR-145 Antagonism Rescue Cigarette Smoke- and TGF-?-Mediated CFTR Expression.
Cystic Fibrosis
A new 9-alkyladenine-cyclic methylglyoxal diadduct activates wt- and F508del-cystic fibrosis transmembrane conductance regulator (CFTR) in vitro and in vivo.
Cystic Fibrosis
A new approach for identifying non-pathogenic mutations. An analysis of the cystic fibrosis transmembrane regulator gene in normal individuals.
Cystic Fibrosis
A new complex allele of the CFTR gene partially explains the variable phenotype of the L997F mutation.
Cystic Fibrosis
A new compound heterozygous CFTR mutation in a Chinese family with cystic fibrosis.
Cystic Fibrosis
A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect.
Cystic Fibrosis
A new frameshift mutation 460delG in exon 4 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
A new insertion/deletion of the cystic fibrosis transmembrane conductance regulator gene accounts for 3.4% of cystic fibrosis mutations in sardinia: implications for population screening.
Cystic Fibrosis
A new missense mutation (R1283M) in exon 20 of the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
A new missense mutation G1249E in exon 20 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
A new missense mutation G126D in exon 4 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
A new model for fish ion regulation: identification of ionocytes in freshwater- and seawater-acclimated medaka (Oryzias latipes).
Cystic Fibrosis
A new model of cystic fibrosis pathology: lack of transport of glutathione and its thiocyanate conjugates.
Cystic Fibrosis
A new polymorphism in exon 7 of the cystic fibrosis transmembrane regulator (CFTR) gene.
Cystic Fibrosis
A new role for heat shock factor 27 in the pathophysiology of Clostridium difficile toxin B.
Cystic Fibrosis
A new stochastic tridimensional model of neonatal rat spinal motoneuron for investigating compartmentalization of neuronal conductances and their influence on firing.
Cystic Fibrosis
A New Targeted CFTR Mutation Panel Based on Next-Generation Sequencing Technology.
Cystic Fibrosis
A nonolfactory shark adenosine receptor activates CFTR with unique pharmacology and structural features.
Cystic Fibrosis
A nonsense mutation (R1158X) and a splicing mutation (3849 + 4A----G) in exon 19 of the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
A Novel Case of Diabetic Muscle Necrosis in a Patient with Cystic Fibrosis-Related Diabetes.
Cystic Fibrosis
A Novel Chimeric Adenoassociated Virus 2/Human Bocavirus 1 Parvovirus Vector Efficiently Transduces Human Airway Epithelia.
Cystic Fibrosis
A Novel Cystic Fibrosis Gene Mutation C.4242+1G>C in an Omani Patient: A Case Report.
Cystic Fibrosis
A novel donor splice site in intron 11 of the CFTR gene, created by mutation 1811+1.6kbA-->G, produces a new exon: high frequency in Spanish cystic fibrosis chromosomes and association with severe phenotype.
Cystic Fibrosis
A novel ER J-protein DNAJB12 accelerates ER-associated degradation of membrane proteins including CFTR.
Cystic Fibrosis
A novel exon duplication of the cystic fibrosis transmembrane conductance regulator in a patient presenting with adult-onset recurrent pancreatitis.
Cystic Fibrosis
A novel exon in the cystic fibrosis transmembrane conductance regulator gene activated by the nonsense mutation E92X in airway epithelial cells of patients with cystic fibrosis.
Cystic Fibrosis
A novel extract SB-300 from the stem bark latex of Croton lechleri inhibits CFTR-mediated chloride secretion in human colonic epithelial cells.
Cystic Fibrosis
A novel fluorescent sensor for measurement of CFTR function by flow cytometry.
Cystic Fibrosis
A Novel G542X CFTR Rat Model of Cystic Fibrosis Is Sensitive to Nonsense Mediated Decay.
Cystic Fibrosis
A novel hemizygous loss-of-function mutation in ADGRG2 causes male infertility with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
A novel mammalian ER-located J-protein, DNAJB14, can accelerate ERAD of misfolded membrane proteins.
Cystic Fibrosis
A novel missense mutation A1081P in the cystic fibrosis transmembrane conductance regulator (CFTR) gene identified in a Laotian patient with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
A novel missense mutation D513G in exon 10 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene identified in a French CBAVD patient. Mutations in brief no. 175. Online.
Cystic Fibrosis
A novel missense mutation in exon 16 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene identified in CBAVD patients.
Cystic Fibrosis
A novel missense mutation P1290S at exon-20 of the CFTR gene in a Portuguese patient with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
A Novel Missense Mutation, R1283S, of the Cystic Fibrosis Transmembrane Conductance Regulator Gene in a 47-Year-Old African-American Patient.
Cystic Fibrosis
A novel model for the first nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
A novel mutation (-195C>A) in the promoter region of CFTR gene is associated with Chinese Congenital Bilateral Absence of Vas Deferens (CBAVD).
Cystic Fibrosis
A novel mutation (M1V) in the translation initiation codon of the cystic fibrosis transmembrane conductance regulator gene, in three CF chromosomes of Italian origin.
Cystic Fibrosis
A novel mutation detected by temporal temperature gradient gel electrophoresis led to the confirmative prenatal diagnosis of a Hispanic CF family.
Cystic Fibrosis
A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations.
Cystic Fibrosis
A novel mutation in the cystic fibrosis transmembrane conductance regulator gene in an Indian patient with idiopathic chronic pancreatitis: a case report.
Cystic Fibrosis
A novel natural product compound enhances cAMP-regulated chloride conductance of cells expressing CFTR[delta]F508.
Cystic Fibrosis
A novel nonsense mutation, S434X, in exon 9 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
A novel nonsense mutation, S466Xa in exon 10 of the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
A novel nonsense mutation, W846XI (amber termination), in exon 14a of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
A novel PDZ protein regulates the activity of guanylyl cyclase C, the heat-stable enterotoxin receptor.
Cystic Fibrosis
A novel procedure for the efficient purification of the cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
A novel quality control compartment derived from the endoplasmic reticulum.
Cystic Fibrosis
A novel regulatory role for tissue transglutaminase in epithelial-mesenchymal transition in cystic fibrosis.
Cystic Fibrosis
A novel role and mechanism of cystic fibrosis transmembrane conductance regulator in bisphenol A-induced prostate cancer.
Cystic Fibrosis
A novel role of protein tyrosine kinase2 in mediating chloride secretion in human airway epithelial cells.
Cystic Fibrosis
A novel splice mutation, 4006-1G>A, in intron 21 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
A novel splice site mutation in the first exon of the cystic fibrosis transmembrane regulator (CFTR) gene identified in a CBAVD patient.
Cystic Fibrosis
A novel treatment of cystic fibrosis acting on-target: cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR.
Cystic Fibrosis
A nuclear factor that binds purine-rich, single-stranded oligonucleotides derived from S1-sensitive elements upstream of the CFTR gene and the MUC1 gene.
Cystic Fibrosis
A pathogenic role for cystic fibrosis transmembrane conductance regulator in celiac disease.
Cystic Fibrosis
A Patient With Pancreas Divisum, Recurrent Acute Pancreatitis, and Homozygosity for the Cystic Fibrosis Transmembrane Regulator-Associated Protein 5T Allele.
Cystic Fibrosis
A PDZ-binding motif is essential but not sufficient to localize the C terminus of CFTR to the apical membrane.
Cystic Fibrosis
A PDZ-interacting domain in CFTR is an apical membrane polarization signal.
Cystic Fibrosis
A Peptide Nucleic Acid (PNA) Masking the miR-145-5p Binding Site of the 3'UTR of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) mRNA Enhances CFTR Expression in Calu-3 Cells.
Cystic Fibrosis
A Peptide Nucleic Acid against MicroRNA miR-145-5p Enhances the Expression of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Calu-3 Cells.
Cystic Fibrosis
A Peptide-Nucleic Acid Targeting miR-335-5p Enhances Expression of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene with the Possible Involvement of the CFTR Scaffolding Protein NHERF1.
Cystic Fibrosis
A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease.
Cystic Fibrosis
A phase 1 study, in cystic fibrosis patients, of the safety, toxicity, and biological efficacy of a single administration of a replication deficient, recombinant adenovirus carrying the cDNA of the normal cystic fibrosis transmembrane conductance regulator gene in the lung.
Cystic Fibrosis
A phase I study of adenovirus-mediated transfer of the human cystic fibrosis transmembrane conductance regulator gene to a lung segment of individuals with cystic fibrosis.
Cystic Fibrosis
A phase I study of aerosolized administration of tgAAVCF to cystic fibrosis subjects with mild lung disease.
Cystic Fibrosis
A phase II, double-blind, randomized, placebo-controlled clinical trial of tgAAVCF using maxillary sinus delivery in patients with cystic fibrosis with antrostomies.
Cystic Fibrosis
A physiologically-motivated model of cystic fibrosis liquid and solute transport dynamics across primary human nasal epithelia.
Cystic Fibrosis
A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function.
Cystic Fibrosis
A pilot study of the effect of gentamicin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations.
Cystic Fibrosis
A placebo-controlled study of liposome-mediated gene transfer to the nasal epithelium of patients with cystic fibrosis.
Cystic Fibrosis
A Plant-Derived Hydrolysable Tannin Inhibits CFTR Chloride Channel: A Potential Treatment of Diarrhea.
Cystic Fibrosis
A plant-derived hydrolysable tannin inhibits CFTR chloride channel: a potential treatment of diarrhea.
Cystic Fibrosis
A plethora of cardiac chloride conductances: molecular diversity or a related gene family.
Cystic Fibrosis
A possible role for intracellular GSH in spontaneous reaction of a cysteine (T338C) engineered into the Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
A potential strategy for reducing cysts in autosomal dominant polycystic kidney disease with a CFTR corrector.
Cystic Fibrosis
A potentiator induces conformational changes on the recombinant CFTR nucleotide binding domains in solution.
Cystic Fibrosis
A principal role for the proteasome in endoplasmic reticulum-associated degradation of misfolded intracellular cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
A proinflammatory, antiapoptotic phenotype underlies the susceptibility to acute pancreatitis in cystic fibrosis transmembrane regulator (-/-) mice.
Cystic Fibrosis
A protein sequence that can encode native structure by disfavoring alternate conformations.
Cystic Fibrosis
A quantitative multistandard reverse transcriptase-polymerase chain reaction assay of the cystic fibrosis transmembrane conductance regulator: its usefulness in studying efficiency of gene transfer.
Cystic Fibrosis
A Randomized Clinical Trial of Metformin to Treat Autosomal Dominant Polycystic Kidney Disease.
Cystic Fibrosis
A randomized placebo-controlled trial of miglustat in cystic fibrosis based on nasal potential difference.
Cystic Fibrosis
A rapid, efficient, and sensitive assay for simultaneous detection of multiple cystic fibrosis mutations.
Cystic Fibrosis
A Rare Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mutation Associated With Typical Cystic Fibrosis in an Arab Child.
Cystic Fibrosis
A rare DNA variant in exon 15 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
A rare mutation in cystic fibrosis transmembrane conductance regulator gene in a recurrent pancreatitis patient without respiratory symptoms.
Cystic Fibrosis
A rat parotid gland cell line, Par-C10, exhibits neurotransmitter-regulated transepithelial anion secretion.
Cystic Fibrosis
A recombinant peptide model of the first nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator: comparison of wild-type and delta F508 mutant forms.
Cystic Fibrosis
A recombinant polypeptide model of the second nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator functions as an active ATPase, GTPase and adenylate kinase.
Cystic Fibrosis
A recombinant polypeptide model of the second predicted nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator is a GTP-binding protein.
Cystic Fibrosis
A regulatory element in intron 1 of the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
A regulatory role of polycystin-1 on cystic fibrosis transmembrane conductance regulator plasma membrane expression.
Cystic Fibrosis
A Review of Trikafta: Triple Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulator Therapy.
Cystic Fibrosis
A Review on the Use of Cystic Fibrosis Transmembrane Conductance Regulator Gene Modulators in Pediatric Patients.
Cystic Fibrosis
A role for CFTR in the elevation of glutathione levels in the lung by oral glutathione administration.
Cystic Fibrosis
A role for the cystic fibrosis transmembrane conductance regulator in the nitric oxide-dependent release of Cl(-) from acidic organelles in amacrine cells.
Cystic Fibrosis
A SAXS-based ensemble model of the native and phosphorylated regulatory domain of the CFTR.
Cystic Fibrosis
A Scientific Rationale for Using Cystic Fibrosis Transmembrane Conductance Regulator Therapeutics in COVID-19 Patients.
Cystic Fibrosis
A second dose of a CFTR cDNA-liposome complex is as effective as the first dose in restoring cAMP-dependent chloride secretion to null CF mice trachea.
Cystic Fibrosis
A sensitive, real-time, RNA-specific PCR method for the detection of recombinant AAV-CFTR vector expression.
Cystic Fibrosis
A sequence upstream of canonical PDZ-binding motif within CFTR COOH-terminus enhances NHERF1 interaction.
Cystic Fibrosis
A sequence variation in intron 17B of the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
A short segment of the R domain of cystic fibrosis transmembrane conductance regulator contains channel stimulatory and inhibitory activities that are separable by sequence modification.
Cystic Fibrosis
A simplified cyclic adenosine monophosphate-mediated sweat rate test for quantitative measure of cystic fibrosis transmembrane regulator (CFTR) function.
Cystic Fibrosis
A single amino acid substitution in CFTR converts ATP to an inhibitory ligand.
Cystic Fibrosis
A single conductance pore for chloride ions formed by two cystic fibrosis transmembrane conductance regulator molecules.
Cystic Fibrosis
A small molecule modulator interacts directly with deltaF508-CFTR to modify its ATPase activity and conformational stability.
Cystic Fibrosis
A small molecule that binds to an ATPase domain of Hsc70 promotes membrane trafficking of mutant cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
A specific cystic fibrosis mutation (T3381) associated with the phenotype of isolated hypotonic dehydration.
Cystic Fibrosis
A splicing mutation in intron 16 of the cystic fibrosis transmembrane conductance regulator gene, associated with severe disease, is common on Reunion Island.
Cystic Fibrosis
A stable ATP binding to the nucleotide binding domain is important for reliable gating cycle in an ABC transporter CFTR.
Cystic Fibrosis
A Stable Human-Cell System Overexpressing Cystic Fibrosis Transmembrane Conductance Regulator Recombinant Protein at the Cell Surface.
Cystic Fibrosis
A study of the mechanism of the release of ATP from rat cortical astroglial cells evoked by activation of glutamate receptors.
Cystic Fibrosis
A survey of detergents for the purification of stable, active human cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
A Survey of the Common Mutations and IVS8-Tn Polymorphism of Cystic Fibrosis Transmembrane Conductance Regulator Gene in Infertile Men with Nonobstructive Azoospermia and CBAVD in Iranian Population
Cystic Fibrosis
A survey of the prevalence, management and outcome of infants with an inconclusive diagnosis following newborn bloodspot screening for cystic fibrosis (CRMS/CFSPID) in six Italian centres.
Cystic Fibrosis
A synonymous codon change alters the drug sensitivity of ?F508 cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
A synonymous single nucleotide polymorphism in DeltaF508 CFTR alters the secondary structure of the mRNA and the expression of the mutant protein.
Cystic Fibrosis
A synthetic channel-forming peptide induces Cl(-) secretion: modulation by Ca(2+)-dependent K(+) channels.
Cystic Fibrosis
A synthetic chloride channel restores chloride conductance in human cystic fibrosis epithelial cells.
Cystic Fibrosis
A Tannic Acid-Based Medical Food, Cesinex(®), Exhibits Broad-Spectrum Antidiarrheal Properties: A Mechanistic and Clinical Study.
Cystic Fibrosis
A termination mutation (2143delT) in the CFTR gene of German cystic fibrosis patients.
Cystic Fibrosis
A topological switch in CFTR modulates channel activity and sensitivity to unfolding.
Cystic Fibrosis
A transformed human tracheal gland cell line, MM-39, that retains serous secretory functions.
Cystic Fibrosis
A two-domain model for the R domain of the cystic fibrosis transmembrane conductance regulator based on sequence similarities.
Cystic Fibrosis
A Unified View of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gating: Combining the Allosterism of a Ligand-gated Channel with the Enzymatic Activity of an ATP-binding Cassette (ABC) Transporter.
Cystic Fibrosis
A unique subset of rat and human intestinal villus cells express the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
A universal array-based multiplexed test for cystic fibrosis carrier screening.
Cystic Fibrosis
A variable dinucleotide repeat in the CFTR gene contributes to phenotype diversity by forming RNA secondary structures that alter splicing.
Cystic Fibrosis
A yeast artificial chromosome contig encompassing the cystic fibrosis locus.
Cystic Fibrosis
A yeast metal resistance protein similar to human cystic fibrosis transmembrane conductance regulator (CFTR) and multidrug resistance-associated protein.
Cystic Fibrosis
A yeast phenomic model for the gene interaction network modulating CFTR-?F508 protein biogenesis.
Cystic Fibrosis
A1 receptor antagonist 8-cyclopentyl-1,3-dipropylxanthine selectively activates chloride efflux from human epithelial and mouse fibroblast cell lines expressing the cystic fibrosis transmembrane regulator delta F508 mutation.
Cystic Fibrosis
A2 adenosine receptors in Mongolian gerbil middle ear epithelium and their regulation of Cl- secretion.
Cystic Fibrosis
A2B adenosine receptors regulate the mucus clearance component of the lung's innate defense system.
Cystic Fibrosis
AAV exploits subcellular stress associated with inflammation, endoplasmic reticulum expansion, and misfolded proteins in models of cystic fibrosis.
Cystic Fibrosis
ABC1, an ATP binding cassette transporter required for phagocytosis of apoptotic cells, generates a regulated anion flux after expression in Xenopus laevis oocytes.
Cystic Fibrosis
ABCC7/CFTR Expression Is Associated with the Clinical Course of Ulcerative Colitis Patients.
Cystic Fibrosis
Aberrant CFTR-dependent HCO3- transport in mutations associated with cystic fibrosis.
Cystic Fibrosis
Aberrant GSH reductase and NOX activities concur with defective CFTR to pro-oxidative imbalance in cystic fibrosis airways.
Cystic Fibrosis
Ability of adenovirus vectors containing different CFTR transcriptional cassettes to correct ion transport defects in CF cells.
Cystic Fibrosis
Ablation of internalization signals in the carboxyl-terminal tail of the cystic fibrosis transmembrane conductance regulator enhances cell surface expression.
Cystic Fibrosis
Abnormal expression of the cystic fibrosis transmembrane regulator in chronic sinusitis in cystic fibrosis and non-cystic fibrosis patients.
Cystic Fibrosis
Abnormal ion content, hydration and granule expansion of the secretory granules from cystic fibrosis airway glandular cells.
Cystic Fibrosis
Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia.
Cystic Fibrosis
Abnormal n-6 fatty acid metabolism in cystic fibrosis contributes to pulmonary symptoms.
Cystic Fibrosis
Abnormal Rab11-Rab8-vesicles cluster in enterocytes of patients with microvillus inclusion disease.
Cystic Fibrosis
Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes.
Cystic Fibrosis
Abnormally enhanced cystic fibrosis transmembrane conductance regulator-mediated apoptosis in endometrial cells contributes to impaired embryo implantation in controlled ovarian hyperstimulation.
Cystic Fibrosis
Abnormally up-regulated cystic fibrosis transmembrane conductance regulator expression and uterine fluid accumulation contribute to Chlamydia trachomatis-induced female infertility.
Cystic Fibrosis
Abolition of Ca2+-mediated intestinal anion secretion and increased stool dehydration in mice lacking the intermediate conductance Ca2+-dependent K+ channel Kcnn4.
Cystic Fibrosis
Absence of CFTR is associated with pleiotropic effects on mucins in mouse gallbladder epithelial cells.
Cystic Fibrosis
Absence of the cystic fibrosis transmembrane regulator (Cftr) from myeloid-derived cells slows resolution of inflammation and infection.
Cystic Fibrosis
Absence of typical unfolded protein response in primary cultured cystic fibrosis airway epithelial cells.
Cystic Fibrosis
ABSOLUTE CONFIGURATION AND BIOLOGICAL PROPERTIES OF ENANTIOMERS OF CFTR INHIBITOR BPO-27.
Cystic Fibrosis
Abundant off-target edits from site-directed RNA editing can be reduced by nuclear localization of the editing enzyme.
Cystic Fibrosis
AC6 is the major adenylate cyclase forming a diarrheagenic protein complex with cystic fibrosis transmembrane conductance regulator in cholera.
Cystic Fibrosis
Academy of Nutrition and Dietetics: 2020 Cystic Fibrosis Evidence Analysis Center Evidence-Based Nutrition Practice Guideline.
Cystic Fibrosis
Acceleration of glutathione efflux and inhibition of gamma-glutamyltranspeptidase sensitize metastatic B16 melanoma cells to endothelium-induced cytotoxicity.
Cystic Fibrosis
Accessory protein facilitated CFTR-CFTR interaction, a molecular mechanism to potentiate the chloride channel activity.
Cystic Fibrosis
Accumulation of azithromycin and roxithromycin in tracheal epithelial fetal cell lines expressing wild type or mutated cystic fibrosis transmembrane conductance regulator protein (CFTR).
Cystic Fibrosis
Accumulation of ceramide in the trachea and intestine of cystic fibrosis mice causes inflammation and cell death.
Cystic Fibrosis
Accumulation of metals in GOLD4 COPD lungs is associated with decreased CFTR levels.
Cystic Fibrosis
Acid-stimulated duodenal bicarbonate secretion involves a CFTR-mediated transport pathway in mice.
Cystic Fibrosis
Acidic Submucosal Gland pH and Elevated Protein Concentration Produce Abnormal Cystic Fibrosis Mucus.
Cystic Fibrosis
Acidification-dependent activation of CD1d-restricted natural killer T cells is intact in cystic fibrosis.
Cystic Fibrosis
Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis and Other Diseases of Mucus Clearance.
Cystic Fibrosis
Acquired defects in CFTR-dependent ?-adrenergic sweat secretion in chronic obstructive pulmonary disease.
Cystic Fibrosis
Actin directly interacts with different membrane channel proteins and influences channel activities: AQP2 as a model.
Cystic Fibrosis
Actin filament organization is required for proper cAMP-dependent activation of CFTR.
Cystic Fibrosis
Actions of adenosine A1 and A2 receptor antagonists on CFTR antibody-inhibited beta-adrenergic mucin secretion response.
Cystic Fibrosis
Actions of genistein on cystic fibrosis transmembrane conductance regulator channel gating. Evidence for two binding sites with opposite effects.
Cystic Fibrosis
Activating cystic fibrosis transmembrane conductance regulator channels with pore blocker analogs.
Cystic Fibrosis
Activation mechanisms for the cystic fibrosis transmembrane conductance regulator protein involve direct binding of cAMP.
Cystic Fibrosis
Activation of a CFTR-mediated chloride current in a rabbit corneal epithelial cell line.
Cystic Fibrosis
Activation of AMP-activated kinase as a strategy for managing autosomal dominant polycystic kidney disease.
Cystic Fibrosis
Activation of AMP-activated protein kinase by a plant-derived dihydroisosteviol in human intestinal epithelial cell.
Cystic Fibrosis
Activation of AMP-activated protein kinase reduces cAMP-mediated epithelial chloride secretion.
Cystic Fibrosis
Activation of AMPK inhibits cholera toxin stimulated chloride secretion in human and murine intestine.
Cystic Fibrosis
Activation of an adenosine 3',5'-cyclic monophosphate-dependent Cl- conductance in response to neurohormonal stimuli in mouse endometrial epithelial cells: the role of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Activation of apical CFTR and basolateral Ca(2+)-activated K+ channels by tetramethylpyrazine in Caco-2 cell line.
Cystic Fibrosis
Activation of cAMP-dependent C1- currents in guinea-pig paneth cells without relevant evidence for CFTR expression.
Cystic Fibrosis
Activation of cardiac chloride conductance by the tyrosine kinase inhibitor, genistein.
Cystic Fibrosis
Activation of CFTR by the Flavonoid Quercetin: Potential Use as a Biomarker of {Delta}F508 CFTR Rescue.
Cystic Fibrosis
Activation of CFTR Cl(-) channel by tyrphostins via a protein tyrosine kinase-independent pathway in forskolin-stimulated renal epithelial A6 cells.
Cystic Fibrosis
Activation of CFTR trafficking and gating by vasoactive intestinal peptide in human bronchial epithelial cells.
Cystic Fibrosis
Activation of Cl- currents by intracellular chloride in fibroblasts stably expressing the human cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Activation of constitutive androstane receptor inhibits intestinal CFTR-mediated chloride transport.
Cystic Fibrosis
Activation of cystic fibrosis transmembrane conductance regulator in rat epididymal epithelium by genistein.
Cystic Fibrosis
Activation of deltaF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX.
Cystic Fibrosis
Activation of endogenous deltaF508 cystic fibrosis transmembrane conductance regulator by phosphodiesterase inhibition.
Cystic Fibrosis
Activation of G551D CFTR channel with MPB-91: regulation by ATPase activity and phosphorylation.
Cystic Fibrosis
Activation of intestinal Cl- secretion by lubiprostone requires the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells.
Cystic Fibrosis
Activation of the cystic fibrosis transmembrane conductance regulator by cGMP in the human colonic cancer cell line, Caco-2.
Cystic Fibrosis
Activation of the cystic fibrosis transmembrane regulator by cyclic AMP is not correlated with inhibition of endocytosis.
Cystic Fibrosis
Activation of transgene expression by early region 4 is responsible for a high level of persistent transgene expression from adenovirus vectors in vivo.
Cystic Fibrosis
Activation of wild-type and deltaF508-CFTR by phosphodiesterase inhibitors through cAMP-dependent and -independent mechanisms.
Cystic Fibrosis
Active-Site Flexibility and Substrate Specificity in a Bacterial Virulence Factor: Crystallographic Snapshots of an Epoxide Hydrolase.
Cystic Fibrosis
Activity and inhibition of prostasin and matriptase on apical and basolateral surfaces of human airway epithelial cells.
Cystic Fibrosis
Acute appendicitis mimicking intestinal obstruction in a patient with cystic fibrosis.
Cystic Fibrosis
Acute inhibition of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel by thyroid hormones involves multiple mechanisms.
Cystic Fibrosis
Acute lung injury edema fluid decreases net fluid transport across human alveolar epithelial type II cells.
Cystic Fibrosis
Acute pancreatitis in association with Campylobacter jejuni-associated diarrhea in a 15-year-old with CFTR mutations: is there a link?
Cystic Fibrosis
Acute Recurrent and Chronic Pancreatitis as Initial Manifestations of Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders.
Cystic Fibrosis
Acute regulation of the SLC26A3 congenital chloride diarrhoea anion exchanger (DRA) expressed in Xenopus oocytes.
Cystic Fibrosis
Acute responses of non-human primates to airway delivery of an adenovirus vector containing the human cystic fibrosis transmembrane conductance regulator cDNA.
Cystic Fibrosis
Acylpeptide hydrolase inhibition as targeted strategy to induce proteasomal down-regulation.
Cystic Fibrosis
Adapting in situ polymerase chain reaction for genotyping of cells in suspension.
Cystic Fibrosis
Adapting Proteostasis and Autophagy for Controlling the Pathogenesis of Cystic Fibrosis Lung Disease.
Cystic Fibrosis
Additional disruption of the ClC-2 Cl(-) channel does not exacerbate the cystic fibrosis phenotype of cystic fibrosis transmembrane conductance regulator mouse models.
Cystic Fibrosis
Adenine nucleotide-induced activation of adenosine A(2B) receptors expressed in Xenopus laevis oocytes: involvement of a rapid and localized adenosine formation by ectonucleotidases.
Cystic Fibrosis
Adeno-associated virus (AAV)-mediated gene transfer in respiratory epithelium and submucosal gland cells in human fetal tracheal organ culture.
Cystic Fibrosis
Adenocarcinoma of the gastrointestinal tract in lung transplanted patients with cystic fibrosis: case series and review of the literature.
Cystic Fibrosis
Adenosine activates a2b receptors and enhances chloride secretion in kidney inner medullary collecting duct cells.
Cystic Fibrosis
Adenosine and its nucleotides activate wild-type and R117H CFTR through an A2B receptor-coupled pathway.
Cystic Fibrosis
Adenosine inhibits arginine vasopressin-stimulated chloride secretion in a mouse IMCD cell line (mIMCD-K2).
Cystic Fibrosis
Adenosine receptors and phosphodiesterase inhibitors stimulate Cl- secretion in Calu-3 cells.
Cystic Fibrosis
Adenosine receptors in rat and human pancreatic ducts stimulate chloride transport.
Cystic Fibrosis
Adenosine regulation of cystic fibrosis transmembrane conductance regulator through prostenoids in airway epithelia.
Cystic Fibrosis
Adenosine triphosphate release and purinergic (P2) receptor-mediated secretion in small and large mouse cholangiocytes.
Cystic Fibrosis
Adenosine triphosphate-binding cassette superfamily transporter gene expression in severe male infertility.
Cystic Fibrosis
Adenosine triphosphate-dependent asymmetry of anion permeation in the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Adenoviral vector expressing ICP47 inhibits adenovirus-specific cytotoxic T lymphocytes in nonhuman primates.
Cystic Fibrosis
Adenovirus-mediated augmentation of cell transfection with unmodified plasmid vectors.
Cystic Fibrosis
Adenovirus-mediated gene transfer to ciliated airway epithelia requires prolonged incubation time.
Cystic Fibrosis
Adenovirus-mediated gene transfer transiently corrects the chloride transport defect in nasal epithelia of patients with cystic fibrosis.
Cystic Fibrosis
Adenovirus-mediated persistent cystic fibrosis transmembrane conductance regulator expression in mouse airway epithelium.
Cystic Fibrosis
Adenovirus-mediated transfer of the CFTR gene to lung of nonhuman primates: toxicity study.
Cystic Fibrosis
Adenovirus?mediated overexpression of cystic fibrosis transmembrane conductance regulator enhances invasiveness and motility of serous ovarian cancer cells.
Cystic Fibrosis
Adenylate Cyclase 1 Links Calcium Signaling to CFTR-Dependent Cytosolic Chloride Elevations in Chick Amacrine Cells.
Cystic Fibrosis
Adenylyl cyclase 6 expression is essential for cholera toxin-induced diarrhea.
Cystic Fibrosis
Adherence to cystic fibrosis transmembrane conductance regulator (CFTR) modulators: analysis of a national specialty pharmacy database.
Cystic Fibrosis
ADP inhibits function of the ABC transporter cystic fibrosis transmembrane conductance regulator via its adenylate kinase activity.
Cystic Fibrosis
Adrenaline-induced colonic K+ secretion is mediated via KCa1.1 (BK) channel.
Cystic Fibrosis
Advances in Cell and Gene-based Therapies for Cystic Fibrosis Lung Disease.
Cystic Fibrosis
Aerosol administration of a recombinant adenovirus expressing CFTR to cystic fibrosis patients: a phase I clinical trial.
Cystic Fibrosis
Aerosol-mediated delivery of recombinant adenovirus to the airways of nonhuman primates.
Cystic Fibrosis
Aerosolized Bovine Lactoferrin Counteracts Infection, Inflammation and Iron Dysbalance in A Cystic Fibrosis Mouse Model of Pseudomonas aeruginosa Chronic Lung Infection.
Cystic Fibrosis
Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors.
Cystic Fibrosis
Affinity purification of insoluble recombinant fusion proteins containing glutathione-S-transferase.
Cystic Fibrosis
After damage of large bile ducts by gamma-aminobutyric acid, small ducts replenish the biliary tree by amplification of calcium-dependent signaling and de novo acquisition of large cholangiocyte phenotypes.
Cystic Fibrosis
After the Celebrations: Lessons from the New Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy.
Cystic Fibrosis
Aggregation of misfolded proteins can be a selective process dependent upon peptide composition.
Cystic Fibrosis
Agonist-induced coordinated trafficking of functionally related transport proteins for water and ions in cholangiocytes.
Cystic Fibrosis
Agonists that stimulate secretion promote the recruitment of CFTR into membrane lipid microdomains.
Cystic Fibrosis
Airway acidification initiates host defense abnormalities in cystic fibrosis mice.
Cystic Fibrosis
Airway epithelial cell exposure to distinct e-cigarette liquid flavorings reveals toxicity thresholds and activation of CFTR by the chocolate flavoring 2,5-dimethypyrazine.
Cystic Fibrosis
Airway epithelial cells-Functional links between CFTR and anoctamin dependent Cl(-) secretion.
Cystic Fibrosis
Airway epithelial CFTR mRNA expression in cystic fibrosis patients after repetitive administration of a recombinant adenovirus.
Cystic Fibrosis
Airway epithelial control of Pseudomonas aeruginosa infection in cystic fibrosis.
Cystic Fibrosis
Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies.
Cystic Fibrosis
Airway Epithelial Wounds in Rhesus Monkey Generate Ionic Currents That Guide Cell Migration to Promote Healing.
Cystic Fibrosis
Airway gene transfer in mouse nasal-airways: importance of identification of epithelial type for assessment of gene transfer.
Cystic Fibrosis
Airway hyperresponsiveness in FVB/N delta F508 cystic fibrosis transmembrane conductance regulator mice.
Cystic Fibrosis
Airway inflammation and infection in congenital bilateral absence of the vas deferens.
Cystic Fibrosis
Airway microbiota and pathogen abundance in age-stratified cystic fibrosis patients.
Cystic Fibrosis
Airway microenvironment alterations and pathogen growth in cystic fibrosis.
Cystic Fibrosis
Airway nitric oxide levels in cystic fibrosis patients are related to a polymorphism in the neuronal nitric oxide synthase gene.
Cystic Fibrosis
Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis.
Cystic Fibrosis
Airway surface liquid homeostasis in cystic fibrosis: pathophysiology and therapeutic targets.
Cystic Fibrosis
Airways in cystic fibrosis are acidified: detection by exhaled breath condensate.
Cystic Fibrosis
AKAP proteins anchor cAMP-dependent protein kinase to KvLQT1/IsK channel complex.
Cystic Fibrosis
Alcohol disrupts levels and function of the cystic fibrosis transmembrane conductance regulator to promote development of pancreatitis.
Cystic Fibrosis
Alcoholic pancreatitis and polymorphisms of the variable length polythymidine tract in the cystic fibrosis gene.
Cystic Fibrosis
Alignment of transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Cystic Fibrosis
Allele frequency for Cystic fibrosis in Indians vis-a/-vis global populations.
Cystic Fibrosis
Allergic Bronchopulmonary Aspergillosis in Children with Cystic Fibrosis: An Update on the Newest Diagnostic Tools and Therapeutic Approaches.
Cystic Fibrosis
Alpha-1 Antitrypsin-A Target for MicroRNA-Based Therapeutic Development for Cystic Fibrosis.
Cystic Fibrosis
Alpha-aminoazaheterocyclic-methylglyoxal adducts do not inhibit cystic fibrosis transmembrane conductance regulator chloride channel activity.
Cystic Fibrosis
Alpha1-antitrypsin deficiency alleles and the Taq-I G-->A allele in cystic fibrosis lung disease.
Cystic Fibrosis
ALTERATION IN THE CYTOKINE SECRETION PATTERN IN T CELLS OF PATIENTS WITH CYSTIC FIBROSIS CAUSED BY DNA METHYLTRANSFERASE INHIBITOR 5-AZACITIDINE.
Cystic Fibrosis
Alteration of CFTR transmembrane span integration by disease-causing mutations.
Cystic Fibrosis
Alteration of Membrane Cholesterol Content Plays a Key Role in Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Channel Activity.
Cystic Fibrosis
Alteration of protein function by a silent polymorphism linked to tRNA abundance.
Cystic Fibrosis
Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway.
Cystic Fibrosis
Alterations of the Nasopharyngeal Microbiota in Infants with Cystic Fibrosis. Cystic Fibrosis Transmembrane Conductance Regulator and Antibiotic Effects.
Cystic Fibrosis
Altered beta-adrenergic and muscarinic response of CFTR Cl- current in dialyzed cardiac myocytes.
Cystic Fibrosis
Altered channel gating mechanism for CFTR inhibition by a high-affinity thiazolidinone blocker.
Cystic Fibrosis
Altered cyclic expression of epithelial Na+ channel subunits and cystic fibrosis transmembrane conductance regulator in mouse endometrium by a low sodium diet.
Cystic Fibrosis
Altered eicosanoid metabolism in the cystic fibrosis mouse small intestine.
Cystic Fibrosis
Altered expression of Na(+)/K(+)-ATPase and other osmoregulatory genes in the gills of euryhaline animals in response to salinity transfer: A meta-analysis of 59 quantitative PCR studies over 10years.
Cystic Fibrosis
Altered ion transport by thyroid epithelia fromCFTR-/- pigs suggests mechanisms for hypothyroidism in Cystic Fibrosis.
Cystic Fibrosis
Altered pHi regulation in 3T3/CFTR clones and their chemotherapeutic drug-selected derivatives.
Cystic Fibrosis
Altered protein folding may be the molecular basis of most cases of cystic fibrosis.
Cystic Fibrosis
Altered respiratory epithelial cell cytokine production in cystic fibrosis.
Cystic Fibrosis
Altered sulfate transport via anion exchange in CFPAC is corrected by retrovirus-mediated CFTR gene transfer.
Cystic Fibrosis
Altering intracellular pH reveals the kinetic basis of intraburst gating in the CFTR Cl(-) channel.
Cystic Fibrosis
Altering Metabolic Profiles of Drugs by Precision Deuteration 2: Discovery of a Deuterated Analog of Ivacaftor with Differentiated Pharmacokinetics for Clinical Development.
Cystic Fibrosis
Alternate translation initiation codons can create functional forms of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Alternating access to the transmembrane domain of the ATP-binding cassette protein cystic fibrosis transmembrane conductance regulator (ABCC7).
Cystic Fibrosis
Alternative splicing of a previously unidentified CFTR exon introduces an in-frame stop codon 5' of the R region.
Cystic Fibrosis
Alternative splicing of intron 23 of the human cystic fibrosis transmembrane conductance regulator gene resulting in a novel exon and transcript coding for a shortened intracytoplasmic C terminus.
Cystic Fibrosis
Ambroxol-induced modification of ion transport in human airway Calu-3 epithelia.
Cystic Fibrosis
Amiloride-sensitive fluid resorption in NCI-H441 lung epithelia depends on an apical Cl(-) conductance.
Cystic Fibrosis
Amino acid residues lining the chloride channel of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Amino acid substitutions in the sixth transmembrane domain of P-glycoprotein alter multidrug resistance.
Cystic Fibrosis
Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.
Cystic Fibrosis
Ammonia exposure affects the mRNA and protein expression levels of certain Rhesus glycoproteins in the gills of climbing perch.
Cystic Fibrosis
AMP-activated protein kinase and nitric oxide regulate the glucose sensitivity of ventromedial hypothalamic glucose-inhibited neurons.
Cystic Fibrosis
AMPK Agonists Ameliorate Sodium and Fluid Transport and Inflammation in CF Airway Epithelial Cells.
Cystic Fibrosis
Amplification of CFTR exon 9 sequences to multiple locations in the human genome.
Cystic Fibrosis
Amplifiers co-translationally enhance CFTR biosynthesis via PCBP1-mediated regulation of CFTR mRNA.
Cystic Fibrosis
Amplifying dinucleotide microsatellite loci from bone and tooth samples of up to 5000 years of age: more inconsistency than usefulness.
Cystic Fibrosis
An "ex vivo model" contributing to the diagnosis and evaluation of new drugs in cystic fibrosis.
Cystic Fibrosis
An "Unlikely" Pair: The Antimicrobial Synergy of Polymyxin B in Combination with the Cystic Fibrosis Transmembrane Conductance Regulator Drugs KALYDECO and ORKAMBI.
Cystic Fibrosis
An Adult Mouse Model of Vibrio cholerae-induced Diarrhea for Studying Pathogenesis and Potential Therapy of Cholera.
Cystic Fibrosis
An alternate pathway of cAMP-stimulated Cl secretion across the NKCC1-null murine duodenum.
Cystic Fibrosis
An anion channel in guinea pig gallbladder epithelial cells is highly permeable to HCO(-)(3).
Cystic Fibrosis
An apical PDZ protein anchors the cystic fibrosis transmembrane conductance regulator to the cytoskeleton.
Cystic Fibrosis
An electrostatic interaction at the tetrahelix bundle promotes phosphorylation-dependent cystic fibrosis transmembrane conductance regulator (CFTR) channel opening.
Cystic Fibrosis
An element in intron 1 of the CFTR gene augments intestinal expression in vivo.
Cystic Fibrosis
An energy-dependent maturation step is required for release of the cystic fibrosis transmembrane conductance regulator from early endoplasmic reticulum biosynthetic machinery.
Cystic Fibrosis
An exonic splicing enhancer offsets the atypical GU-rich 3' splice site of human apolipoprotein A-II exon 3.
Cystic Fibrosis
An improved method to obtain highly differentiated monolayers of human bronchial epithelial cells.
Cystic Fibrosis
An Innovative Protocol for Metaproteomic Analyses of Microbial Pathogens in Cystic Fibrosis Sputum.
Cystic Fibrosis
An integrated mathematical epithelial cell model for airway surface liquid regulation by mechanical forces.
Cystic Fibrosis
An international randomized multicenter comparison of nasal potential difference techniques.
Cystic Fibrosis
An intrinsic adenylate kinase activity regulates gating of the ABC transporter CFTR.
Cystic Fibrosis
An intronic polypyrimidine-rich element downstream of the donor site modulates cystic fibrosis transmembrane conductance regulator exon 9 alternative splicing.
Cystic Fibrosis
An inwardly rectifying potassium channel in apical membrane of Calu-3 cells.
Cystic Fibrosis
An MBL2 haplotype and ABCB4 variants modulate the risk of liver disease in cystic fibrosis patients: a multicentre study.
Cystic Fibrosis
An unstable transmembrane segment in the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Analysis of 30 known cystic fibrosis mutations: 10 mutations account for 27% of non-delta F508 chromosomes in southern France.
Cystic Fibrosis
Analysis of 31 CFTR mutations by polymerase chain reaction/oligonucleotide ligation assay in a pilot screening of 4476 newborns for cystic fibrosis.
Cystic Fibrosis
Analysis of a DNase I hypersensitive site located -20.9 kb upstream of the CFTR gene.
Cystic Fibrosis
Analysis of alternative splicing patterns in the cystic fibrosis transmembrane conductance regulator gene using mRNA derived from lymphoblastoid cells of cystic fibrosis patients.
Cystic Fibrosis
Analysis of c.3499+200TA(7_56) and D7S523 Microsatellites Linked to Cystic Fibrosis Transmembrane Regulator.
Cystic Fibrosis
Analysis of CA/GT microsatellite polymorphism in IVS8 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene: a study of Italian CF families.
Cystic Fibrosis
Analysis of CFTR Gene Mutations in Children with Cystic Fibrosis, First Report from North-East of Iran.
Cystic Fibrosis
Analysis of CFTR Gene Variants in Idiopathic Bronchiectasis in Serbian Children.
Cystic Fibrosis
Analysis of cis and trans Requirements for DNA Replication at the Right-End Hairpin of the Human Bocavirus 1 Genome.
Cystic Fibrosis
Analysis of Conventional and Unconventional Trafficking of CFTR and Other Membrane Proteins.
Cystic Fibrosis
Analysis of cystic fibrosis transmembrane conductance regulator gene mutations in patients with congenital absence of the uterus and vagina.
Cystic Fibrosis
Analysis of cystic fibrosis transmembrane regulator and azoospermia factor polymorphisms in infertile men in relation to other abnormalities.
Cystic Fibrosis
Analysis of cystic fibrosis-associated P67L CFTR illustrates barriers to personalized therapeutics for orphan diseases.
Cystic Fibrosis
Analysis of DNase-I-hypersensitive sites at the 3' end of the cystic fibrosis transmembrane conductance regulator gene (CFTR).
Cystic Fibrosis
ANALYSIS OF LOCAL IMMUNITY INDICATORS OF THE ORAL CAVITY AND DEGREE OF GINGIVITIS DEPENDING ON MUTATION OF CFTR GENE IN CHILDREN WITH CYSTIC FIBROSIS.
Cystic Fibrosis
Analysis of long-range interactions in primary human cells identifies cooperative CFTR regulatory elements.
Cystic Fibrosis
Analysis of microsatellites by direct blotting electrophoresis and chemiluminescence detection.
Cystic Fibrosis
Analysis of polymerase chain reaction-amplified DNA products by mass spectrometry using matrix-assisted laser desorption and electrospray: current status.
Cystic Fibrosis
Analysis of the 27 exons and flanking regions of the cystic fibrosis gene: 40 different mutations account for 91.2% of the mutant alleles in southern France.
Cystic Fibrosis
Analysis of the CFTR gene confirms the high genetic heterogeneity of the Spanish population: 43 mutations account for only 78% of CF chromosomes.
Cystic Fibrosis
Analysis of the CFTR gene in Iranian cystic fibrosis patients: identification of eight novel mutations.
Cystic Fibrosis
Analysis of the CFTR gene in Turkish cystic fibrosis patients: identification of three novel mutations (3172delAC, P1013L and M1028I).
Cystic Fibrosis
Analysis of the entire coding region of the cystic fibrosis transmembrane regulator gene in neonatal hypertrypsinaemia with normal sweat test.
Cystic Fibrosis
Analysis of the entire coding region of the cystic fibrosis transmembrane regulator gene in idiopathic pancreatitis.
Cystic Fibrosis
Analysis of the human pancreatic secretory trypsin inhibitor (PSTI) gene mutations in Japanese patients with chronic pancreatitis.
Cystic Fibrosis
Analysis of the intron-exon organization of the human multidrug-resistance protein gene (MRP) and alternative splicing of its mRNA.
Cystic Fibrosis
Analysis of the localization of STE6/CFTR chimeras in a Saccharomyces cerevisiae model for the cystic fibrosis defect CFTR delta F508.
Cystic Fibrosis
Analysis of the whole CFTR coding regions and splice junctions in azoospermic men with congenital bilateral aplasia of epididymis or vas deferens.
Cystic Fibrosis
Analytical validation considerations of multiplex mass-spectrometry-based proteomic platforms for measuring protein biomarkers.
Cystic Fibrosis
Anchored PDE4 regulates chloride conductance in wild-type and ?F508-CFTR human airway epithelia.
Cystic Fibrosis
Andrological findings in infertile men with two (biallelic) CFTR mutations: results of a multicentre study in Germany and Austria comprising 71 patients.
Cystic Fibrosis
Angiotensin II evokes calcium-mediated signaling events in isolated dog pancreatic epithelial cells.
Cystic Fibrosis
Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences.
Cystic Fibrosis
Anion secretion by the inner medullary collecting duct. Evidence for involvement of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Annexin A5 increases the cell surface expression and the chloride channel function of the DeltaF508-cystic fibrosis transmembrane regulator.
Cystic Fibrosis
Annexin V is directly involved in cystic fibrosis transmembrane conductance regulator's chloride channel function.
Cystic Fibrosis
Anoctamin 1 induces calcium-activated chloride secretion and proliferation of renal cyst-forming epithelial cells.
Cystic Fibrosis
Anoctamin 6 Contributes to Cl- Secretion in Accessory Cholera Enterotoxin (Ace)-stimulated Diarrhea: AN ESSENTIAL ROLE FOR PHOSPHATIDYLINOSITOL 4,5-BISPHOSPHATE (PIP2) SIGNALING IN CHOLERA.
Cystic Fibrosis
Anoctamin 6 is an essential component of the outwardly rectifying chloride channel.
Cystic Fibrosis
Anoctamins support calcium-dependent chloride secretion by facilitating calcium signaling in adult mouse intestine.
Cystic Fibrosis
ANP and CNP activate CFTR expressed in Xenopus laevis oocytes by direct activation of PKA.
Cystic Fibrosis
Antagonistic Regulation of CFTR Cell Surface Expression by Protein Kinases WNK4 and Spleen Tyrosine Kinase.
Cystic Fibrosis
Antagonists of myo-inositol 3,4,5,6-tetrakisphosphate allow repeated epithelial chloride secretion.
Cystic Fibrosis
Anti-Infectives Restore ORKAMBI® Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of P. aeruginosa.
Cystic Fibrosis
Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis.
Cystic Fibrosis
Antibiotic exposure and interpersonal variance mask the effect of ivacaftor on respiratory microbiota composition.
Cystic Fibrosis
Antibodies to the CFTR modulate the turgor pressure of guard cell protoplasts via slow anion channels.
Cystic Fibrosis
Antibody against a cystic fibrosis transmembrane conductance regulator-derived synthetic peptide inhibits anion currents in human colonic cell line T84.
Cystic Fibrosis
Antidiarrheal Action of Bacillus subtilis CU1 CNCM I-2745 and Lactobacillus plantarum CNCM I-4547 in Mice.
Cystic Fibrosis
Antidiarrheal efficacy of a quinazolin CFTR inhibitor on human intestinal epithelial cell and in mouse model of cholera.
Cystic Fibrosis
Antihypertensive 1,4-dihydropyridines as correctors of the cystic fibrosis transmembrane conductance regulator channel gating defect caused by cystic fibrosis mutations.
Cystic Fibrosis
Antimicrobial Properties of Mesenchymal Stem Cells: Therapeutic Potential for Cystic Fibrosis Infection, and Treatment.
Cystic Fibrosis
Antineutrophil cytoplasmic autoantibodies (ANCA) in children with cystic fibrosis.
Cystic Fibrosis
Antioxidant imbalance in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice.
Cystic Fibrosis
Antisense oligodeoxynucleotide to the cystic fibrosis transmembrane conductance regulator inhibits cyclic AMP-activated but not calcium-activated cell volume reduction in a human pancreatic duct cell line.
Cystic Fibrosis
Antisense oligodeoxynucleotides to the cystic fibrosis transmembrane conductance regulator inhibit cAMP-activated but not calcium-activated chloride currents.
Cystic Fibrosis
Antisense oligonucleotide eluforsen is safe and improves respiratory symptoms in F508DEL cystic fibrosis.
Cystic Fibrosis
Antisense oligonucleotide to PKC-epsilon alters cAMP-dependent stimulation of CFTR in Calu-3 cells.
Cystic Fibrosis
Antisense oligonucleotides to CFTR confer a cystic fibrosis phenotype on B lymphocytes.
Cystic Fibrosis
Antisera preparation and epitope mapping of a recombinant protein comprising three peptide fragments of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
AP2 ? modulates cystic fibrosis transmembrane conductance regulator function in the human intestine.
Cystic Fibrosis
Apical adenosine regulates basolateral Ca2+-activated potassium channels in human airway Calu-3 epithelial cells.
Cystic Fibrosis
Apical and basolateral ATP stimulates tracheal epithelial chloride secretion via multiple purinergic receptors.
Cystic Fibrosis
Apical localization of the coxsackie-adenovirus receptor by glycosyl-phosphatidylinositol modification is sufficient for adenovirus-mediated gene transfer through the apical surface of human airway epithelia.
Cystic Fibrosis
Apparent affinity of CFTR for ATP is increased by continuous kinase activity.
Cystic Fibrosis
Apparent homozygosity of a novel frame shift mutation in the CFTR gene because of a large deletion.
Cystic Fibrosis
Apparent Homozygosity of p.Phe508del in CFTR due to a Large Gene Deletion of Exons 4-11.
Cystic Fibrosis
Applicability of different antibodies for immunohistochemical localization of CFTR in sweat glands from healthy controls and from patients with cystic fibrosis.
Cystic Fibrosis
Application of DETECTER, an evolutionary genomic tool to analyze genetic variation, to the cystic fibrosis gene family.
Cystic Fibrosis
Application of high-resolution single-channel recording to functional studies of cystic fibrosis mutants.
Cystic Fibrosis
Application of mass spectrometry to study proteomics and interactomics in cystic fibrosis.
Cystic Fibrosis
Application of pyrosequencing to the identification of sequence variations in the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Application of the luminex LabMAP in rapid screening for mutations in the cystic fibrosis transmembrane conductance regulator gene: A pilot study
Cystic Fibrosis
Applying Cystic Fibrosis Transmembrane Conductance Regulator Genetics and CFTR2 Data to Facilitate Diagnoses.
Cystic Fibrosis
Aquagenic Wrinkling of the Palm: A Rare Diagnostic Clue of Cystic Fibrosis and the Response to CFTR-Modulating Therapy.
Cystic Fibrosis
Aquagenic wrinkling of the palms in cystic fibrosis and the cystic fibrosis carrier state: a casecontrol study.
Cystic Fibrosis
Aquaporin 3 cloned from Xenopus laevis is regulated by the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Aquaporin-4 as a molecular partner of cystic fibrosis transmembrane conductance regulator in rat Sertoli cells.
Cystic Fibrosis
Aquaporin-9 is expressed in rat Sertoli cells and interacts with the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Aqueous cigarette smoke extract induces a voltage-dependent inhibition of CFTR expressed in Xenopus oocytes.
Cystic Fibrosis
AR-13 reduces antibiotic-resistant bacterial burden in cystic fibrosis phagocytes and improves cystic fibrosis transmembrane conductance regulator function.
Cystic Fibrosis
Arachidonic acid release by ionomycin and phorbol ester is similar in C127 epithelial cells expressing wild-type or mutated (delta F508) cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Architectural proteins CTCF and cohesin have distinct roles in modulating the higher order structure and expression of the CFTR locus.
Cystic Fibrosis
Architecture of the cystic fibrosis transmembrane conductance regulator protein and structural changes associated with phosphorylation and nucleotide binding.
Cystic Fibrosis
Arg352 is a major determinant of charge selectivity in the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Arrhythmia and Sudden Death Associated with Elevated Cardiac Chloride Channel Activity.
Cystic Fibrosis
Arsenic promotes ubiquitinylation and lysosomal degradation of cystic fibrosis transmembrane conductance regulator (CFTR) chloride channels in human airway epithelial cells.
Cystic Fibrosis
Arsenite regulates Cystic Fibrosis Transmembrane Conductance Regulator and P-glycoprotein: evidence of pathway independence.
Cystic Fibrosis
Arylaminobenzoate block of the cardiac cyclic AMP-dependent chloride current.
Cystic Fibrosis
Aspergillus colonization and antifungal immunity in cystic fibrosis patients.
Cystic Fibrosis
Aspergillus fumigatus generates an enhanced Th2-biased immune response in mice with defective cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Aspergillus-induced superoxide production by cystic fibrosis phagocytes is associated with disease severity.
Cystic Fibrosis
Aspirin and some other nonsteroidal anti-inflammatory drugs inhibit cystic fibrosis transmembrane conductance regulator protein gene expression in T-84 cells.
Cystic Fibrosis
Assembly and Functional Analysis of an S/MAR Based Episome with the Cystic Fibrosis Transmembrane Conductance Regulator Gene.
Cystic Fibrosis
Assembly and misassembly of cystic fibrosis transmembrane conductance regulator: folding defects caused by deletion of F508 occur before and after the calnexin-dependent association of membrane spanning domain (MSD) 1 and MSD2.
Cystic Fibrosis
Assembly and trafficking of a multiprotein ROMK (Kir 1.1) channel complex by PDZ interactions.
Cystic Fibrosis
Assembly of gap junction channels: mechanism, effects of calmodulin antagonists and identification of connexin oligomerization determinants.
Cystic Fibrosis
Assembly of signaling complexes by the sodium-hydrogen exchanger regulatory factor family of PDZ-containing proteins.
Cystic Fibrosis
Assessing effects of inhaled antibiotics in adults with non-cystic fibrosis bronchiectasis--experiences from recent clinical trials.
Cystic Fibrosis
Assessment of acquired mucociliary clearance defects using micro-optical coherence tomography.
Cystic Fibrosis
Assessment of Aspergillus sensitization or persistent carriage as a factor in lung function impairment in cystic fibrosis patients.
Cystic Fibrosis
Assessment of CFTR function in rectal biopsies for the diagnosis of cystic fibrosis.
Cystic Fibrosis
Assessment of chloride secretion in human nasal epithelial cells by X-ray microanalysis.
Cystic Fibrosis
Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation.
Cystic Fibrosis
Assessment of dental status and oral hygiene in the study population of cystic fibrosis patients in the Podlasie province.
Cystic Fibrosis
Assessment of hospitalization rates, factors associated with hospitalization and in-patient mortality in pediatric patients with cystic fibrosis.
Cystic Fibrosis
Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cells.
Cystic Fibrosis
Assisted reproduction technology for patients with congenital bilateral absence of vas deferens.
Cystic Fibrosis
Assisting PNA transport through cystic fibrosis human airway epithelia with biodegradable hybrid lipid-polymer nanoparticles.
Cystic Fibrosis
Association Analyses of Genetic Polymorphisms of GSTM1, GSTT1, NQO1, NAT2, LPL, PRSS1, PSTI, and CFTR With Chronic Alcoholic Pancreatitis in Japan.
Cystic Fibrosis
Association between Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations and Susceptibility for Childhood Asthma in Korea.
Cystic Fibrosis
Association between serum oncofetal antigens CA 19-9 and CA 125 and clinical status in patients with cystic fibrosis.
Cystic Fibrosis
Association of CFTR gene mutation with bronchial asthma and its severity in Indian children: a case-control study.
Cystic Fibrosis
Association of Common Haplotypes of Surfactant Protein A1 and A2 (SFTPA1 and SFTPA2) Genes with Severity of Lung Disease in Cystic Fibrosis.
Cystic Fibrosis
Association of cystic fibrosis genetic modifiers with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
Association of cystic fibrosis transmembrane conductance regulator (CFTR) mutation/variant/haplotype and tumor necrosis factor (TNF) promoter polymorphism in hyperlipidemic pancreatitis.
Cystic Fibrosis
Association of cystic fibrosis transmembrane conductance regulator and protein phosphatase 2C.
Cystic Fibrosis
Association of cystic fibrosis transmembrane conductance regulator gene variants with acute lung injury in African American children with pneumonia.
Cystic Fibrosis
Association of cystic fibrosis transmembrane conductance regulator with epithelial sodium channel subunits carrying Liddle's syndrome mutations.
Cystic Fibrosis
Association of cystic fibrosis with abnormalities in fatty acid metabolism.
Cystic Fibrosis
Association of domains within the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Association of IVS6A GATT polymorphism of CFTR gene with cystic fibrosis: first study in CF and normal Tunisian population.
Cystic Fibrosis
Association of M470V polymorphism of CFTR gene with variability of clinical expression of asthma: Case-report study.
Cystic Fibrosis
Association of pancreas divisum and recurrent acute pancreatitis with the IVS8-5T-12TG allele of the CFTR gene and CFTR dysfunction.
Cystic Fibrosis
Association of polymorphisms in genes of factors involved in regulation of splicing of cystic fibrosis transmembrane conductance regulator mRNA with acute respiratory distress syndrome in children with pneumonia.
Cystic Fibrosis
Association of S549N and IVS8-5T Splice Variants with Bronchial Asthma and Its Severity in Indian Children.
Cystic Fibrosis
Association of the cystic fibrosis transmembrane regulator with CAL: structural features and molecular dynamics.
Cystic Fibrosis
Association of TNF-? Gene Variants With Clinical Manifestation of Cystic Fibrosis Patients of Iranian Azeri Turkish Ethnicity.
Cystic Fibrosis
Associations between peak oxygen uptake, lung function, and bronchiectasis in children with cystic fibrosis in the era of CFTR modulators.
Cystic Fibrosis
Asthma and COPD in cystic fibrosis intron-8 5T carriers. A population-based study.
Cystic Fibrosis
Asthma and pulmonary function abnormalities in heterozygotes for cystic fibrosis transmembrane regulator gene mutations.
Cystic Fibrosis
Asymmetric 4-Aryl-1,4-dihydropyridines Potentiate Mutant Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Cystic Fibrosis
Asymmetric structure of the cystic fibrosis transmembrane conductance regulator chloride channel pore suggested by mutagenesis of the twelfth transmembrane region.
Cystic Fibrosis
Asymptomatic cystic fibrosis diagnosed in an adult evaluated for hematuria.
Cystic Fibrosis
Ataluren (PTC124) Induces CFTR Protein Expression and Activity in Children with Nonsense Mutation Cystic Fibrosis.
Cystic Fibrosis
Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis.
Cystic Fibrosis
Ataluren/ivacaftor combination therapy: Two N-of-1 trials in cystic fibrosis patients with nonsense mutations.
Cystic Fibrosis
Atomic model of human cystic fibrosis transmembrane conductance regulator: membrane-spanning domains and coupling interfaces.
Cystic Fibrosis
Atomic Structure of the Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
ATP alters current fluctuations of cystic fibrosis transmembrane conductance regulator: evidence for a three-state activation mechanism.
Cystic Fibrosis
ATP and AMP Mutually Influence Their Interaction with the ATP-binding Cassette (ABC) Adenylate Kinase Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) at Separate Binding Sites.
Cystic Fibrosis
ATP binding cassette modulators control abscisic acid-regulated slow anion channels in guard cells
Cystic Fibrosis
ATP depletion induces a loss of respiratory epithelium functional integrity and down-regulates CFTR (cystic fibrosis transmembrane conductance regulator) expression.
Cystic Fibrosis
ATP hydrolysis by a CFTR domain: pharmacology and effects of G551D mutation.
Cystic Fibrosis
ATP hydrolysis-dependent asymmetry of the conformation of CFTR channel pore.
Cystic Fibrosis
ATP release triggered by activation of the Ca2+-activated K+ channel in human airway Calu-3 cells.
Cystic Fibrosis
ATP-dependent activation of K(Ca) and ROMK-type K(ATP) channels in human submandibular gland ductal cells.
Cystic Fibrosis
ATP-Dependent Signaling in Simulations of a Revised Model of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Cystic Fibrosis
ATP-dependent transport systems in bacteria and humans: relevance to cystic fibrosis and multidrug resistance.
Cystic Fibrosis
ATP-independent CFTR channel gating and allosteric modulation by phosphorylation.
Cystic Fibrosis
ATP-induced Cl- secretion with suppressed Na+ absorption in rabbit tracheal epithelium.
Cystic Fibrosis
Atrial natriuretic peptide modulates cystic fibrosis transmembrane conductance regulator chloride channel expression in rat proximal colon and human intestinal epithelial cells.
Cystic Fibrosis
Attenuation of Phosphorylation-dependent Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Disease-causing Mutations at the Transmission Interface.
Cystic Fibrosis
Atypical sinusitis in adults must lead to looking for cystic fibrosis and primary ciliary dyskinesia.
Cystic Fibrosis
Augmentation of S-Nitrosoglutathione Controls Cigarette Smoke-Induced Inflammatory-Oxidative Stress and Chronic Obstructive Pulmonary Disease-Emphysema Pathogenesis by Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function.
Cystic Fibrosis
Auto-obliteration of maxillary sinuses through osteoneogenesis in children with cystic fibrosis: A possible new way to reduce morbidity.
Cystic Fibrosis
Autophagy Augmentation to Alleviate Immune Response Dysfunction, and Resolve Respiratory and COVID-19 Exacerbations.
Cystic Fibrosis
Autosomal dominant polycystic kidney disease coexisting with cystic fibrosis.
Cystic Fibrosis
Azaspiracids Increase Mitochondrial Dehydrogenases Activity in Hepatocytes: Involvement of Potassium and Chloride Ions.
Cystic Fibrosis
Azithromycin and tezacaftor/ivacaftor is associated with first-degree heart block in an adult with cystic fibrosis.
Cystic Fibrosis
Azithromycin fails to reduce inflammation in cystic fibrosis airway epithelial cells.
Cystic Fibrosis
Bacterial overgrowth in the cystic fibrosis transmembrane conductance regulator null mouse small intestine.
Cystic Fibrosis
Bacterial periplasmic permeases as model systems for multidrug resistance (MDR) and the cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
Bacterial periplasmic permeases as model systems for the superfamily of traffic ATPases, including the multidrug resistance protein and the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Bacterial Sphingomyelinase is a State-Dependent Inhibitor of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR).
Cystic Fibrosis
Bafilomycin A1-sensitive pathway is required for the maturation of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
BAG-1 stabilizes mutant F508del-CFTR in a ubiquitin-like-domain-dependent manner.
Cystic Fibrosis
BAG-2 acts as an inhibitor of the chaperone-associated ubiquitin ligase CHIP.
Cystic Fibrosis
Basal chloride currents in murine airway epithelial cells: modulation by CFTR.
Cystic Fibrosis
Basal expression of the cystic fibrosis transmembrane conductance regulator gene is dependent on protein kinase A activity.
Cystic Fibrosis
Basal nucleotide levels, release, and metabolism in normal and cystic fibrosis airways.
Cystic Fibrosis
Basal Serum Cortisol and Testosterone/Cortisol Ratio Are Related to Rate of Na+ Lost During Exercise in Elite Soccer Players.
Cystic Fibrosis
Base treatment corrects defects due to misfolding of mutant cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Basic protocol for transepithelial nasal potential difference measurements.
Cystic Fibrosis
Basolateral chloride transporters in autosomal dominant polycystic kidney disease.
Cystic Fibrosis
Bcl-2 suppresses sarcoplasmic/endoplasmic reticulum Ca2+-ATPase expression in cystic fibrosis airways: role in oxidant-mediated cell death.
Cystic Fibrosis
Benefit-Risk Assessment of Plecanatide in the Treatment of Chronic Idiopathic Constipation.
Cystic Fibrosis
Benign and Deleterious Cystic Fibrosis Transmembrane Conductance Regulator Mutations Identified by Sequencing in Positive Cystic Fibrosis Newborn Screen Children from California.
Cystic Fibrosis
Benign outcome among positive cystic fibrosis newborn screen children with non-CF-causing variants.
Cystic Fibrosis
Benzo(c)quinolizinium drugs inhibit degradation of Delta F508-CFTR cytoplasmic domain.
Cystic Fibrosis
Benzoflavone activators of the cystic fibrosis transmembrane conductance regulator: towards a pharmacophore model for the nucleotide-binding domain.
Cystic Fibrosis
Benzopyrimido-pyrrolo-oxazine-dione (R)-BPO-27 Inhibits CFTR Chloride Channel Gating by Competition with ATP.
Cystic Fibrosis
Benzopyrimido-pyrrolo-oxazine-dione CFTR inhibitor (R)-BPO-27 for antisecretory therapy of diarrheas caused by bacterial enterotoxins.
Cystic Fibrosis
Beta-adrenergic receptors couple to CFTR chloride channels of intercalated mitochondria-rich cells in the heterocellular toad skin epithelium.
Cystic Fibrosis
beta3-adrenoceptor control the cystic fibrosis transmembrane conductance regulator through a cAMP/protein kinase A-independent pathway.
Cystic Fibrosis
Beyond cystic fibrosis transmembrane conductance regulator (CFTR) single channel kinetics: implications for therapeutic intervention.
Cystic Fibrosis
Beyond cystic fibrosis transmembrane conductance regulator therapy: a perspective on gene therapy and small molecule treatment for cystic fibrosis.
Cystic Fibrosis
Bicarbonate and chloride secretion in Calu-3 human airway epithelial cells.
Cystic Fibrosis
Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype.
Cystic Fibrosis
Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Bicarbonate secretion by rat bile duct brush cells indicated by immunohistochemical localization of CFTR, anion exchanger AE2, Na+/HCO3 -cotransporter, carbonic anhydrase II, Na+/H+ exchangers NHE1 and NHE3, H+/K+-ATPase, and Na+/K+-ATPase.
Cystic Fibrosis
Bicarbonate secretion in the murine gallbladder--lessons for the treatment of cystic fibrosis.
Cystic Fibrosis
Bicarbonate secretion of mouse cholangiocytes involves Na(+)-HCO(3)(-) cotransport in addition to Na(+)-independent Cl(-)/HCO(3)(-) exchange.
Cystic Fibrosis
Bicarbonate-rich choleresis induced by secretin in normal rat is taurocholate-dependent and involves AE2 anion exchanger.
Cystic Fibrosis
Bicarbonate-rich fluid secretion predicted by a computational model of guinea-pig pancreatic duct epithelium.
Cystic Fibrosis
Bilateral carcinoma in situ of the testis and cystic fibrosis transmembrane conductance regulator (CFTR) mutation in an azoospermic patient with late-onset 21beta-hydroxylase deficiency.
Cystic Fibrosis
Bile acids stimulate chloride secretion through CFTR and calcium-activated Cl- channels in Calu-3 airway epithelial cells.
Cystic Fibrosis
Bile-derived organoids from patients with primary sclerosing cholangitis recapitulate their inflammatory immune profile.
Cystic Fibrosis
Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis.
Cystic Fibrosis
Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator.
Cystic Fibrosis
Binding of serum response factor to cystic fibrosis transmembrane conductance regulator CArG-like elements, as a new potential CFTR transcriptional regulation pathway.
Cystic Fibrosis
Binding screen for cystic fibrosis transmembrane conductance regulator correctors finds new chemical matter and yields insights into cystic fibrosis therapeutic strategy.
Cystic Fibrosis
Binding site of activators of the cystic fibrosis transmembrane conductance regulator in the nucleotide binding domains.
Cystic Fibrosis
Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity.
Cystic Fibrosis
Bioactivity-Guided Fractionation of an Antidiarrheal Chinese Herb Rhodiola kirilowii (Regel) Maxim Reveals (-)-Epicatechin-3-Gallate and (-)-Epigallocatechin-3-Gallate as Inhibitors of Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
Biochemical and biophysical identification of cystic fibrosis transmembrane conductance regulator chloride channels as components of endocytic clathrin-coated vesicles.
Cystic Fibrosis
Biochemical basis of the interaction between cystic fibrosis transmembrane conductance regulator (CFTR) and immunoglobulin-like repeats of filamin.
Cystic Fibrosis
Biochemical characterization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis epithelial cells.
Cystic Fibrosis
Biochemical evidence for ATPase activity in CFTR-enriched apical membrane vesicles from tracheal epithelium.
Cystic Fibrosis
Biochemical implications of sequence comparisons of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Biogenesis of CFTR and other polytopic membrane proteins: new roles for the ribosome-translocon complex.
Cystic Fibrosis
Biological and structural basis for Aha1 regulation of Hsp90 ATPase activity in maintaining proteostasis in the human disease cystic fibrosis.
Cystic Fibrosis
Biological Characterization of F508delCFTR Protein Processing by the CFTR Corrector ABBV-2222/GLPG2222.
Cystic Fibrosis
Biophysical characterisation of calumenin as a charged F508del-CFTR folding modulator.
Cystic Fibrosis
Biosynthetic and growth abnormalities are associated with high-level expression of CFTR in heterologous cells.
Cystic Fibrosis
Birth after intracytoplasmic injection of epididymal sperm from a man with congenital bilateral absence of the vas deferens who had a robertsonian translocation.
Cystic Fibrosis
Birth of a normal girl after in vitro fertilization and preimplantation diagnostic testing for cystic fibrosis.
Cystic Fibrosis
Block by MOPS reveals a conformation change in the CFTR pore produced by ATP hydrolysis.
Cystic Fibrosis
Block of CFTR-dependent chloride currents by inhibitors of multidrug resistance-associated proteins.
Cystic Fibrosis
Blood concentrations of pancreatitis associated protein in neonates: relevance to neonatal screening for cystic fibrosis.
Cystic Fibrosis
Blood immunoreactive trypsinogen concentrations are genetically determined in healthy and cystic fibrosis newborns.
Cystic Fibrosis
Blue native/SDS-PAGE analysis reveals reduced expression of the mClCA3 protein in cystic fibrosis knock-out mice.
Cystic Fibrosis
Body composition and weight changes after ivacaftor treatment in adults with cystic fibrosis carrying the G551 D cystic fibrosis transmembrane conductance regulator mutation: A double-blind, placebo-controlled, randomized, crossover study with open-label extension.
Cystic Fibrosis
Bone Cells Differentiation: How CFTR Mutations May Rule the Game of Stem Cells Commitment?
Cystic Fibrosis
Bone disease in cystic fibrosis: new pathogenic insights opening novel therapies.
Cystic Fibrosis
Borderline sweat test: Utility and limits of genetic analysis for the diagnosis of cystic fibrosis.
Cystic Fibrosis
Both CFTR and outwardly rectifying chloride channels contribute to cAMP-stimulated whole cell chloride currents.
Cystic Fibrosis
Both the wild type and a functional isoform of CFTR are expressed in kidney.
Cystic Fibrosis
Bovine AAV transcytosis inhibition by tannic acid results in functional expression of CFTR in vitro and altered biodistribution in vivo.
Cystic Fibrosis
Bowel ultrasound imaging in patients with cystic fibrosis: Relationship with clinical symptoms and CFTR genotype.
Cystic Fibrosis
Branchial ionocyte organization and ion-transport protein expression in juvenile alewives acclimated to freshwater or seawater.
Cystic Fibrosis
Buccal cell DNA analysis in premature and term neonates: screening for mutations of the complete coding region for the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Buccal cell DNA mutation analysis for diagnosis of cystic fibrosis in newborns and infants inaccessible to sweat chloride measurement.
Cystic Fibrosis
Building an understanding of cystic fibrosis on the foundation of ABC transporter structures.
Cystic Fibrosis
Burkholderia cenocepacia-induced delay of acidification and phagolysosomal fusion in cystic fibrosis transmembrane conductance regulator (CFTR)-defective macrophages.
Cystic Fibrosis
Buserelin alleviates chloride transport defect in human cystic fibrosis nasal epithelial cells.
Cystic Fibrosis
Butyrate increases apical membrane CFTR but reduces chloride secretion in MDCK cells.
Cystic Fibrosis
c-Cbl facilitates endocytosis and lysosomal degradation of cystic fibrosis transmembrane conductance regulator in human airway epithelial cells.
Cystic Fibrosis
C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis. A novel class of mutation.
Cystic Fibrosis
C-type natriuretic peptide and its receptor are downregulated in pulmonary epithelium following birth.
Cystic Fibrosis
C-type natriuretic peptide increases chloride permeability in normal and cystic fibrosis airway cells.
Cystic Fibrosis
c.753_754delAG, a novel CFTR mutation found in a Chinese patient with cystic fibrosis: A case report and review of the literature.
Cystic Fibrosis
CA/GT microsatellite alleles within the cystic fibrosis transmembrane conductance regulator (CFTR) gene are not generated by unequal crossingover.
Cystic Fibrosis
Ca2+ and cAMP-activated Cl- conductances mediate Cl- secretion in a mouse renal inner medullary collecting duct cell line.
Cystic Fibrosis
Calcitonin Receptor-Mediated CFTR Activation in Human Intestinal Epithelial Cells.
Cystic Fibrosis
Calcium is not involved in the cAMP-mediated stimulation of Cl- conductance in the apical membrane of Necturus gallbladder epithelium.
Cystic Fibrosis
Calcium Modulated Chloride Pathways Contribute to Chloride Flux in Murine CF-Affected Macrophages.
Cystic Fibrosis
Calcium-activated chloride conductance is not increased in pancreatic duct cells of CF mice.
Cystic Fibrosis
Calcium-activated chloride currents and non-selective cation channels in a novel cystic fibrosis-derived human pancreatic duct cell line.
Cystic Fibrosis
Calcium-pump inhibitors induce functional surface expression of Delta F508-CFTR protein in cystic fibrosis epithelial cells.
Cystic Fibrosis
Calculating expected lung deposition of aerosolized administration of AAV vector in human clinical studies.
Cystic Fibrosis
Calculating posterior cystic fibrosis risk with echogenic bowel and one characterized cystic fibrosis mutation: avoiding pitfalls in the risk calculations.
Cystic Fibrosis
Calnexin Delta 185-520 partially reverses the misprocessing of the Delta F508 cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Calpain inhibition promotes the rescue of F(508)del-CFTR in PBMC from cystic fibrosis patients.
Cystic Fibrosis
Calreticulin negatively regulates the cell surface expression of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Calu-3: a human airway epithelial cell line that shows cAMP-dependent Cl- secretion.
Cystic Fibrosis
cAMP activation of chloride and fluid secretion across the rabbit alveolar epithelium.
Cystic Fibrosis
cAMP and genistein stimulate HCO3- conductance through CFTR in human airway epithelia.
Cystic Fibrosis
cAMP inhibition of murine intestinal Na/H exchange requires CFTR-mediated cell shrinkage of villus epithelium.
Cystic Fibrosis
cAMP regulates cell proliferation and cyst formation in autosomal polycystic kidney disease cells.
Cystic Fibrosis
cAMP regulation of Cl(-) and HCO(-)(3) secretion across rat fetal distal lung epithelial cells.
Cystic Fibrosis
cAMP stimulates CFTR-like Cl- channels and inhibits amiloride-sensitive Na+ channels in mouse CCD cells.
Cystic Fibrosis
cAMP Stimulates SLC26A3 Activity in Human Colon by a CFTR-Dependent Mechanism That Does Not Require CFTR Activity.
Cystic Fibrosis
cAMP stimulation of CFTR-expressing Xenopus oocytes activates a chromanol-inhibitable K+ conductance.
Cystic Fibrosis
cAMP- and Ca2+-independent activation of cystic fibrosis transmembrane conductance regulator channels by phenylimidazothiazole drugs.
Cystic Fibrosis
cAMP- but not Ca(2+)-regulated Cl- conductance in the oviduct is defective in mouse model of cystic fibrosis.
Cystic Fibrosis
cAMP- but not Ca(2+)-regulated Cl- conductance is lacking in cystic fibrosis mice epididymides and seminal vesicles.
Cystic Fibrosis
cAMP-activated Cl channels in CFTR-transfected cystic fibrosis pancreatic epithelial cells.
Cystic Fibrosis
cAMP-activated Cl- channels in primary cultures of spiny dogfish (Squalus acanthias) rectal gland.
Cystic Fibrosis
cAMP-dependent activation of CFTR inhibits the epithelial sodium channel (ENaC) without affecting its surface expression.
Cystic Fibrosis
cAMP-dependent activation of ion conductances in bronchial epithelial cells.
Cystic Fibrosis
cAMP-dependent chloride secretion mediates tubule enlargement and cyst formation by cultured mammalian collecting duct cells.
Cystic Fibrosis
cAMP-dependent exocytosis and vesicle traffic regulate CFTR and fluid transport in rat jejunum in vivo.
Cystic Fibrosis
cAMP-dependent protein kinase-mediated phosphorylation of cystic fibrosis transmembrane conductance regulator residue Ser-753 and its role in channel activation.
Cystic Fibrosis
cAMP-independent activation of CFTR Cl channels by the tyrosine kinase inhibitor genistein.
Cystic Fibrosis
cAMP-independent phosphorylation activation of CFTR by G proteins in native human sweat duct.
Cystic Fibrosis
cAMP-inducible chloride conductance in mouse fibroblast lines stably expressing the human cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
cAMP/protein kinase A activates cystic fibrosis transmembrane conductance regulator for ATP release from rat skeletal muscle during low pH or contractions.
Cystic Fibrosis
Campylobacter jejuni infection suppressed Cl? secretion induced by CFTR activation in T-84 cells.
Cystic Fibrosis
Can a polymorphism in the thalassemia gene and a heterozygote CFTR mutation cause acute pancreatitis?
Cystic Fibrosis
Can Cystic Fibrosis Patients Finally Catch a Breath With Lumacaftor/Ivacaftor?
Cystic Fibrosis
Can intervention in inositol phosphate signalling pathways improve therapy for cystic fibrosis?
Cystic Fibrosis
Can Tangier disease cause male infertility? A case report and an overview on genetic causes of male infertility and hormonal axis involved.
Cystic Fibrosis
Can Targeting Iron Help in Combating Chronic Pseudomonas Infection? A Systematic Review.
Cystic Fibrosis
Capacitance measurements reveal different pathways for the activation of CFTR.
Cystic Fibrosis
Capacitation-associated alkalization in human sperm is differentially controlled at the subcellular level.
Cystic Fibrosis
Capsaicin potentiates wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride-channel currents.
Cystic Fibrosis
Capsaicin-Loaded Chitosan Nanocapsules for wtCFTR-mRNA Delivery to a Cystic Fibrosis Cell Line.
Cystic Fibrosis
Capsaicinoids regulate airway anion transporters through Rho kinase- and cAMP-dependent mechanisms.
Cystic Fibrosis
Carbachol-induced MUC17 endocytosis is concomitant with NHE3 internalization and CFTR membrane recruitment in enterocytes.
Cystic Fibrosis
Carbamazepine-induced sperm disorders can be associated with the altered expressions of testicular KCNJ11/miR-let-7a and spermatozoal CFTR/miR-27a.
Cystic Fibrosis
Carbon monoxide-releasing molecules inhibit the cystic fibrosis transmembrane conductance regulator Cl- channel.
Cystic Fibrosis
Carbon nanofiber-based multiplexed immunosensor for the detection of survival motor neuron 1, cystic fibrosis transmembrane conductance regulator and Duchenne Muscular Dystrophy proteins.
Cystic Fibrosis
Cardiac expression of the cystic fibrosis transmembrane conductance regulator involves novel exon 1 usage to produce a unique amino-terminal protein.
Cystic Fibrosis
Cardiomyocytes with disrupted CFTR function require CaMKII and Ca2+-activated Cl- channel activity to maintain contraction rate.
Cystic Fibrosis
Cardiovascular complications in cystic fibrosis: A review of the literature.
Cystic Fibrosis
Caring for Adolescents with Cystic Fibrosis, in Portugal: The Nurse's Role.
Cystic Fibrosis
Carrier detection and prenatal diagnosis of cystic fibrosis using an intragenic TA-repeat polymorphism.
Cystic Fibrosis
Carvedilol binding to ?2-adrenergic receptors inhibits CFTR-dependent anion secretion in airway epithelial cells.
Cystic Fibrosis
Catalyst-like modulation of transition states for CFTR channel opening and closing: new stimulation strategy exploits nonequilibrium gating.
Cystic Fibrosis
Catamenial haemoptysis in females with cystic fibrosis: a case series with review of management strategies.
Cystic Fibrosis
Catch me if you can! Novel aspects of cadmium transport in mammalian cells.
Cystic Fibrosis
Categories of deltaF508 homozygous cystic fibrosis twin and sibling pairs with distinct phenotypic characteristics.
Cystic Fibrosis
Cationic lipid formulations for intracellular gene delivery of cystic fibrosis transmembrane conductance regulator to airway epithelia.
Cystic Fibrosis
Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis: a double-blind placebo-controlled trial.
Cystic Fibrosis
Cationic lipids for reporter gene and CFTR transfer to rat pulmonary epithelium.
Cystic Fibrosis
Cefoperazone sodium liposomal formulation to mitigate P. aeruginosa biofilm in Cystic fibrosis infection: A QbD approach.
Cystic Fibrosis
Ceftolozane/tazobactam for pulmonary exacerbation in a 63-year-old cystic fibrosis patient with renal insufficiency and an elevated MIC to Pseudomonas aeruginosa.
Cystic Fibrosis
CELF proteins regulate CFTR pre-mRNA splicing: essential role of the divergent domain of ETR-3.
Cystic Fibrosis
Celiac Disease in Patients With Cystic Fibrosis on Ivacaftor: A Case Series.
Cystic Fibrosis
Cell culture models demonstrate that CFTR dysfunction leads to defective fatty acid composition and metabolism.
Cystic Fibrosis
Cell engineering method using fluorogenic oligonucleotide signaling probes and flow cytometry.
Cystic Fibrosis
Cell surface expression and turnover of the alpha-subunit of the epithelial sodium channel.
Cystic Fibrosis
Cell volume response to hyposmotic shock and elevated cAMP in bovine trabecular meshwork cells.
Cystic Fibrosis
Cell Volume-Activated and Volume-Correlated Anion Channels in Mammalian Cells: Their Biophysical, Molecular, and Pharmacological Properties.
Cystic Fibrosis
Cell-based assay for high-throughput quantitative screening of CFTR chloride transport agonists.
Cystic Fibrosis
Cell-Selective Regulation of CFTR Gene Expression: Relevance to Gene Editing Therapeutics.
Cystic Fibrosis
Cell-type-specific long-range looping interactions identify distant regulatory elements of the CFTR gene.
Cystic Fibrosis
Cellular and viral splicing factors can modify the splicing pattern of CFTR transcripts carrying splicing mutations.
Cystic Fibrosis
Cellular ATP release by the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Cellular differentiation is required for cAMP but not Ca(2+)-dependent Cl- secretion in colonic epithelial cells expressing high levels of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Cellular differentiation regulates expression of Cl- transport and cystic fibrosis transmembrane conductance regulator mRNA in human intestinal cells.
Cystic Fibrosis
Cellular distribution and function of ion channels involved in transport processes in rat tracheal epithelium.
Cystic Fibrosis
Cellular localization and activity of Ad-delivered GFP-CFTR in airway epithelial and tracheal cells.
Cystic Fibrosis
Cellular localization of cystic fibrosis transmembrane regulator protein in piglet and mouse intestine.
Cystic Fibrosis
Cellular localization of the cystic fibrosis transmembrane conductance regulator in mouse intestinal tract.
Cystic Fibrosis
Cellular mechanism for potentiation of Ca2+-mediated Cl- secretion by the flavonoid baicalein in intestinal epithelia.
Cystic Fibrosis
Cellular mechanism underlying oxytocin-stimulated Cl- secretion in rat cauda epididymal epithelium.
Cystic Fibrosis
Cellular mechanisms underlying carbon monoxide stimulated anion secretion in rat epididymal epithelium.
Cystic Fibrosis
Cellular Model of TAR DNA-binding Protein 43 (TDP-43) Aggregation Based on Its C-terminal Gln/Asn-rich Region.
Cystic Fibrosis
Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis.
Cystic Fibrosis
Certain background factors exhibit an association with an increased risk for pancreatic calcification among Japanese male alcoholics.
Cystic Fibrosis
CF gene and cystic fibrosis transmembrane conductance regulator expression in autosomal dominant polycystic kidney disease.
Cystic Fibrosis
CF2603/4delT, a new frameshift mutation in exon 13 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
CFTR 5T variant has a low penetrance in females that is partially attributable to its haplotype.
Cystic Fibrosis
CFTR activation in human bronchial epithelial cells by novel benzoflavone and benzimidazolone compounds.
Cystic Fibrosis
CFTR activation suppresses glioblastoma cell proliferation, migration and invasion.
Cystic Fibrosis
CFTR activation: additive effects of stimulatory and inhibitory phosphorylation sites in the R domain.
Cystic Fibrosis
CFTR activator increases intestinal fluid secretion and normalizes stool output in a mouse model of constipation.
Cystic Fibrosis
CFTR and Anoctamin 1 (ANO1) contribute to cAMP amplified exocytosis and insulin secretion in human and murine pancreatic beta-cells.
Cystic Fibrosis
CFTR and calcium-activated chloride channels in primary cultures of human airway gland cells of serous or mucous phenotype.
Cystic Fibrosis
CFTR and calcium-activated chloride currents in pancreatic duct cells of a transgenic CF mouse.
Cystic Fibrosis
CFTR and defective endocytosis: new insights in the renal phenotype of cystic fibrosis.
Cystic Fibrosis
Cftr and ENaC ion channels mediate NaCl absorption in the mouse submandibular gland.
Cystic Fibrosis
CFTR and its key role in in vivo resting and luminal acid-induced duodenal HCO3- secretion.
Cystic Fibrosis
CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship.
Cystic Fibrosis
CFTR anion channel modulates expression of human transmembrane mucin MUC3 through the PDZ protein GOPC.
Cystic Fibrosis
CFTR antisense phosphorothioate oligodeoxynucleotides (S-ODns) induce tracheo-bronchial mucin (TBM) mRNA expression in human airway mucosa.
Cystic Fibrosis
CFTR channel in oocytes from Xenopus laevis and its regulation by xShroom1 protein.
Cystic Fibrosis
CFTR channel insertion to the apical surface in rat duodenal villus epithelial cells is upregulated by VIP in vivo.
Cystic Fibrosis
CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains.
Cystic Fibrosis
CFTR channels expressed in CHO cells do not have detectable ATP conductance.
Cystic Fibrosis
CFTR chloride channel activation by genistein: the role of serine/threonine protein phosphatases.
Cystic Fibrosis
CFTR chloride channel activity modulates the mitochondrial morphology in cultured epithelial cells.
Cystic Fibrosis
CFTR chloride channel drug discovery--inhibitors as antidiarrheals and activators for therapy of cystic fibrosis.
Cystic Fibrosis
CFTR chloride channel in the apical compartments: spatiotemporal coupling to its interacting partners.
Cystic Fibrosis
CFTR chloride channel is a molecular target of the natural cancer preventive agent resveratrol.
Cystic Fibrosis
CFTR Cl- channel and CFTR-associated ATP channel: distinct pores regulated by common gates.
Cystic Fibrosis
CFTR Cl- channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis.
Cystic Fibrosis
CFTR Cl- channel functional regulation by phosphorylation of focal adhesion kinase at tyrosine 407 in osmosensitive ion transporting mitochondria rich cells of euryhaline killifish.
Cystic Fibrosis
CFTR constrains the differentiation from mouse embryonic stem cells to intestine lineage cells.
Cystic Fibrosis
Cftr controls lumen expansion and function of Kupffer's vesicle in zebrafish.
Cystic Fibrosis
CFTR corrector C17 is effective in muscular dystrophy, in vivo proof of concept in LGMDR3.
Cystic Fibrosis
CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells.
Cystic Fibrosis
CFTR Deficiency Affects Glucose Homeostasis via Regulating GLUT4 Plasma Membrane Transportation.
Cystic Fibrosis
CFTR Deletion Confers Mitochondrial Dysfunction and Disrupts Lipid Homeostasis in Intestinal Epithelial Cells.
Cystic Fibrosis
CFTR deltaF508 carrier status, risk of breast cancer before the age of 40 and histological grading in a population-based case-control study.
Cystic Fibrosis
CFTR DeltaF508 mutation has minimal effect on the gene expression profile of differentiated human airway epithelia.
Cystic Fibrosis
CFTR Depletion Confers Hypersusceptibility to Mycobacterium fortuitum in a Zebrafish Model.
Cystic Fibrosis
CFTR depletion results in changes in fatty acid composition and promotes lipogenesis in intestinal Caco 2/15 cells.
Cystic Fibrosis
CFTR disruption impairs cAMP-dependent Cl(-) secretion in primary cultures of mouse cortical collecting ducts.
Cystic Fibrosis
CFTR dysfunction in cystic fibrosis and chronic obstructive pulmonary disease.
Cystic Fibrosis
CFTR Expression Analysis for Subtyping of Human Pancreatic Cancer Organoids.
Cystic Fibrosis
CFTR expression and chloride secretion in polarized immortal human bronchial epithelial cells.
Cystic Fibrosis
CFTR expression and mucin secretion in cultured mouse gallbladder epithelial cells.
Cystic Fibrosis
CFTR Expression But Not Cl- Transport Is Involved in the Stimulatory Effect of Bile Acids on Apical Cl-/HCO3- Exchange Activity in Human Pancreatic Duct Cells.
Cystic Fibrosis
CFTR fails to inhibit the epithelial sodium channel ENaC expressed in Xenopus laevis oocytes.
Cystic Fibrosis
CFTR function and clinical response to modulators parallel nasal epithelial organoid swelling.
Cystic Fibrosis
CFTR Functions as a Tumor Suppressor and Is Regulated by DNA Methylation in Colorectal Cancer.
Cystic Fibrosis
CFTR gating II: Effects of nucleotide binding on the stability of open states.
Cystic Fibrosis
CFTR gene analysis in cystic fibrosis patients: detection of 91% of molecular defects and identification of the novel mutation D979V.
Cystic Fibrosis
CFTR gene mutation spectrum among 735 Iranian patients with cystic fibrosis: A comprehensive systematic review.
Cystic Fibrosis
CFTR gene mutations and asthma in the Norwegian Environment and Childhood Asthma study.
Cystic Fibrosis
CFTR gene mutations and polymorphism are associated with non-obstructive azoospermia: From case-control study.
Cystic Fibrosis
CFTR gene mutations in pancreatitis: Frequency and clinical manifestations in an Austrian patient cohort.
Cystic Fibrosis
CFTR gene mutations--including three novel nucleotide substitutions--and haplotype background in patients with asthma, disseminated bronchiectasis and chronic obstructive pulmonary disease.
Cystic Fibrosis
CFTR gene transfer corrects defective glycoconjugate secretion in human CF epithelial tracheal cells.
Cystic Fibrosis
CFTR genotype and clinical outcomes of adult patients carried as cystic fibrosis disease.
Cystic Fibrosis
CFTR Genotype and Maximal Exercise Capacity in Cystic Fibrosis: A Cross-sectional Study.
Cystic Fibrosis
CFTR haplotype analysis reveals genetic heterogeneity in the etiology of congenital bilateral aplasia of the vas deferens.
Cystic Fibrosis
CFTR haplotypic variability for normal and mutant genes in cystic fibrosis families from southern France.
Cystic Fibrosis
CFTR Heterozygotes Are at Increased Risk of Respiratory Infections: A Population-Based Study.
Cystic Fibrosis
CFTR illegitimate transcription in lymphoid cells: quantification and applications to the investigation of pathological transcripts.
Cystic Fibrosis
CFTR impairment upregulates c-Src activity through IL-1? autocrine signaling.
Cystic Fibrosis
CFTR in a lipid raft-TNFR1 complex modulates gap junctional intercellular communication and IL-8 secretion.
Cystic Fibrosis
CFTR in Calu-3 human airway cells: channel properties and role in cAMP-activated Cl- conductance.
Cystic Fibrosis
CFTR Inactivation by Lentiviral Vector-mediated RNA Interference and CRISPR-Cas9 Genome Editing in Human Airway Epithelial Cells.
Cystic Fibrosis
CFTR induces extracellular acid sensing in Xenopus oocytes which activates endogenous Ca(2+)-activated Cl (-) conductance.
Cystic Fibrosis
CFTR induces the expression of DRA along with Cl(-)/HCO(3)(-) exchange activity in tracheal epithelial cells.
Cystic Fibrosis
CFTR Influences Beta Cell Function and Insulin Secretion Through Non-Cell Autonomous Exocrine-Derived Factors.
Cystic Fibrosis
CFTR inhibition augments NHE3 activity during luminal high CO2 exposure in rat duodenal mucosa.
Cystic Fibrosis
CFTR inhibits the invasion and growth of esophageal cancer cells by inhibiting the expression of NF-?B.
Cystic Fibrosis
CFTR interacts with Hsp90 and regulates the phosphorylation of AKT and ERK1/2 in colorectal cancer cells.
Cystic Fibrosis
CFTR intron 1 increases luciferase expression driven by CFTR 5'-flanking DNA in a yeast artificial chromosome.
Cystic Fibrosis
CFTR involvement in chloride, bicarbonate, and liquid secretion by airway submucosal glands.
Cystic Fibrosis
CFTR is a pattern recognition molecule that extracts Pseudomonas aeruginosa LPS from the outer membrane into epithelial cells and activates NF-kappa B translocation.
Cystic Fibrosis
CFTR is a potential marker for nasopharyngeal carcinoma prognosis and metastasis.
Cystic Fibrosis
CFTR is essential for sperm fertilizing capacity and is correlated with sperm quality in humans.
Cystic Fibrosis
CFTR is involved in membrane endocytosis but not in fluid-phase and receptor-mediated endocytosis in human respiratory epithelial cells.
Cystic Fibrosis
CFTR is involved in the regulation of glucagon secretion in human and rodent alpha cells.
Cystic Fibrosis
CFTR is required for cAMP inhibition of intestinal Na+ absorption in a cystic fibrosis mouse model.
Cystic Fibrosis
CFTR is required for cellular entry and internalization of Chlamydia trachomatis.
Cystic Fibrosis
CFTR is required for maximal transepithelial liquid transport in pig alveolar epithelia.
Cystic Fibrosis
CFTR is required for PKA-regulated ATP sensitivity of Kir1.1 potassium channels in mouse kidney.
Cystic Fibrosis
CFTR is required for the migration of primordial germ cells during zebrafish early embryogenesis.
Cystic Fibrosis
CFTR is restricted to a small population of high expresser cells that provide a forskolin-sensitive transepithelial Cl- conductance in the proximal colon of the possum, Trichosurus vulpecula.
Cystic Fibrosis
CFTR IVS8 Poly-T Variation Affects Severity of Acute Pancreatitis in Women.
Cystic Fibrosis
CFTR Knockdown induces proinflammatory changes in intestinal epithelial cells.
Cystic Fibrosis
CFTR KNOCKDOWN STIMULATES LIPID SYNTHESIS AND TRANSPORT IN INTESTINAL CACO-2/15 CELLS.
Cystic Fibrosis
CFTR localization in native airway cells and cell lines expressing wild-type or F508del-CFTR by a panel of different antibodies.
Cystic Fibrosis
CFTR may play a role in regulated secretion by lymphocytes: a new hypothesis for the pathophysiology of cystic fibrosis.
Cystic Fibrosis
CFTR mediates electrogenic chloride secretion in mouse inner medullary collecting duct (mIMCD-K2) cells.
Cystic Fibrosis
CFTR modulates programmed cell death by decreasing intracellular pH in Chinese hamster lung fibroblasts.
Cystic Fibrosis
CFTR modulates RPS27 gene expression using chloride anion as signaling effector.
Cystic Fibrosis
Cftr Modulates Wnt/?-Catenin Signaling and Stem Cell Proliferation in Murine Intestine.
Cystic Fibrosis
CFTR modulator drug desensitization: Preserving the hope of long term improvement.
Cystic Fibrosis
CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis.
Cystic Fibrosis
CFTR Modulators: Impact on Fertility, Pregnancy, and Lactation in Women with Cystic Fibrosis.
Cystic Fibrosis
CFTR mRNA expression is regulated by an upstream open reading frame and RNA secondary structure in its 5' untranslated region.
Cystic Fibrosis
CFTR Mutation Analysis of a Caucasian Father with Congenital Bilateral Absence of Vas Deferens, a Taiwanese Mother, and Twins Resulting from ICSI Procedure.
Cystic Fibrosis
CFTR mutation enhances Dishevelled degradation and results in impairment of Wnt-dependent hematopoiesis.
Cystic Fibrosis
CFTR mutations and host susceptibility to Pseudomonas aeruginosa lung infection.
Cystic Fibrosis
CFTR mutations impart elevated immune reactivity in a murine model of cystic fibrosis related diabetes.
Cystic Fibrosis
CFTR mutations spectrum and the efficiency of molecular diagnostics in Polish cystic fibrosis patients.
Cystic Fibrosis
CFTR negatively regulates cyclooxygenase-2-PGE(2) positive feedback loop in inflammation.
Cystic Fibrosis
CFTR null mutation altered cAMP-sensitive and swelling-activated Cl- currents in primary cultures of mouse nephron.
Cystic Fibrosis
CFTR polymorphisms of healthy individuals in two Chinese cities--Changchun and Nanjing.
Cystic Fibrosis
CFTR POTENTIATOR VX-770 (IVACAFTOR) OPENS THE DEFECTIVE CHANNEL GATE OF MUTANT CFTR IN A PHOSPHORYLATION-DEPENDENT BUT ATP-INDEPENDENT MANNER.
Cystic Fibrosis
CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR.
Cystic Fibrosis
CFTR prevents neuronal apoptosis following cerebral ischemia reperfusion via regulating mitochondrial oxidative stress.
Cystic Fibrosis
CFTR promotes malignant glioma development via up-regulation of Akt/Bcl2-mediated anti-apoptosis pathway.
Cystic Fibrosis
CFTR protects against vascular inflammation and atherogenesis in apolipoprotein E-deficient mice.
Cystic Fibrosis
CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP.
Cystic Fibrosis
CFTR regulates phagosome acidification in macrophages and alters bactericidal activity.
Cystic Fibrosis
CFTR Regulates the Proliferation, Migration and Invasion of Cervical Cancer Cells by Inhibiting the NF-?B Signalling Pathway.
Cystic Fibrosis
CFTR regulation in human airway epithelial cells requires integrity of the actin cytoskeleton and compartmentalized cAMP and PKA activity.
Cystic Fibrosis
CFTR Regulation of Aquaporin-Mediated Water Transport: A Target in Male Fertility.
Cystic Fibrosis
CFTR Regulation of Intracellular pH and Ceramides is Required for Lung Endothelial Cell Apoptosis.
Cystic Fibrosis
CFTR regulatory region interacts with NBD1 predominantly via multiple transient helices.
Cystic Fibrosis
CFTR silencing in pancreatic ?-cells reveals a functional impact on glucose-stimulated insulin secretion and oxidative stress response.
Cystic Fibrosis
CFTR supports cell death through ROS-dependent activation of TMEM16F (anoctamin 6).
Cystic Fibrosis
CFTR surface expression and chloride currents are decreased by inhibitors of N-WASP and actin polymerization.
Cystic Fibrosis
CFTR targeted therapies: recent advances in cystic fibrosis and possibilities in other diseases of the airways.
Cystic Fibrosis
CFTR Therapeutics Normalize Cerebral Perfusion Deficits in Mouse Models of Heart Failure and Subarachnoid Hemorrhage.
Cystic Fibrosis
CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates.
Cystic Fibrosis
CFTR transcription defects in pancreatic sufficient cystic fibrosis patients with only one mutation in the coding region of CFTR.
Cystic Fibrosis
CFTR transgene expression in primary DeltaF508 epithelial cell cultures from human nasal polyps following gene transfer with cationic phosphonolipids.
Cystic Fibrosis
CFTR transmembrane segments are impaired in their conformational adaptability by a pathogenic loop mutation and dynamically stabilized by Lumacaftor.
Cystic Fibrosis
CFTR upregulates the expression of the basolateral Na(+)-K(+)-2Cl(-) cotransporter in cultured pancreatic duct cells.
Cystic Fibrosis
CFTR's proteolysis protection. The cystic fibrosis transmembrane conductance regulator impedes proteolytic stimulation of the epithelial Na+ channel.
Cystic Fibrosis
CFTR, investigated with the two-electrode voltage-clamp technique: the importance of knowing the series resistance.
Cystic Fibrosis
CFTR, PRSS1 and SPINK1 mutations in the development of pancreatitis in Brazilian patients.
Cystic Fibrosis
CFTR, SPINK1, CTRC and PRSS1 variants in chronic pancreatitis: is the role of mutated CFTR overestimated?
Cystic Fibrosis
CFTR, SPINK1, PRSS1, and CTRC Mutations Are Not Associated With Pancreatic Cancer in German Patients.
Cystic Fibrosis
CFTR-?-catenin interaction regulates mouse embryonic stem cell differentiation and embryonic development.
Cystic Fibrosis
CFTR-deficiency renders mice highly susceptible to cutaneous symptoms during mite infestation.
Cystic Fibrosis
CFTR-deficient pigs display alterations of bone microarchitecture and composition at birth.
Cystic Fibrosis
CFTR-dependent chloride efflux in cystic fibrosis mononuclear cells is increased by ivacaftor therapy.
Cystic Fibrosis
CFTR-dependent membrane insertion is linked to stimulation of the CFTR chloride conductance.
Cystic Fibrosis
CFTR-function and ventilation inhomogeneity in individuals with cystic fibrosis.
Cystic Fibrosis
CFTR-independent ATP release from epithelial cells triggered by mechanical stimuli.
Cystic Fibrosis
CFTR-mediated anion secretion in parathyroid hormone-treated Caco-2 cells is associated with PKA and PI3K phosphorylation but not intracellular pH changes or Na+/K+-ATPase abundance.
Cystic Fibrosis
CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells.
Cystic Fibrosis
CFTR-mediated Cl(-) transport in the acinar and duct cells of rabbit lacrimal gland.
Cystic Fibrosis
CFTR-mediated inhibition of epithelial Na+ conductance in human colon is defective in cystic fibrosis.
Cystic Fibrosis
CFTR-regulated chloride transport at the ocular surface in living mice measured by potential differences.
Cystic Fibrosis
CFTR: Ligand exchange between a permeant anion ([Au(CN)2]-) and an engineered cysteine (T338C) blocks the pore.
Cystic Fibrosis
CFTR: New insights into structure and function and implications for modulation by small molecules.
Cystic Fibrosis
CFTR: the nucleotide binding folds regulate the accessibility and stability of the activated state.
Cystic Fibrosis
cGMP inhibition of type 3 phosphodiesterase is the major mechanism by which C-type natriuretic peptide activates CFTR in the shark rectal gland.
Cystic Fibrosis
cGMP stimulation of cystic fibrosis transmembrane conductance regulator Cl- channels co-expressed with cGMP-dependent protein kinase type II but not type Ibeta.
Cystic Fibrosis
Challenges Facing Airway Epithelial Cell-Based Therapy for Cystic Fibrosis.
Cystic Fibrosis
Challenges in care of adult CF patients--the specialist cystic fibrosis team.
Cystic Fibrosis
Challenges in the use of highly effective modulator treatment for cystic fibrosis.
Cystic Fibrosis
Changes in accessibility of cytoplasmic substances to the pore associated with activation of the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Changes in gene expression in gills of the euryhaline killifish Fundulus heteroclitus after abrupt salinity transfer.
Cystic Fibrosis
Changes in lipid raft proteome upon TNF-? stimulation of cystic fibrosis cells.
Cystic Fibrosis
Changes in the R-region interactions depend on phosphorylation and contribute to PKA and PKC regulation of the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Changes of chloride channels in the lacrimal glands of a rabbit model of Sjögren syndrome.
Cystic Fibrosis
Changing landscape: psychological care in the era of cystic fibrosis transmembrane conductance regulator modulators.
Cystic Fibrosis
Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators.
Cystic Fibrosis
Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop.
Cystic Fibrosis
Channel-lining residues in the M3 membrane-spanning segment of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia.
Cystic Fibrosis
Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell.
Cystic Fibrosis
Chaperoning the maturation of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model.
Cystic Fibrosis
Characterization and localization of the rabbit ocular calcitonin gene-related peptide (CGRP)-receptor component protein (RCP).
Cystic Fibrosis
Characterization of 19 disease-associated missense mutations in the regulatory domain of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Characterization of a 7,8-benzoflavone double effect on CFTR Cl(-) channel activity.
Cystic Fibrosis
Characterization of a critical role for CFTR chloride channels in cardioprotection against ischemia/reperfusion injury.
Cystic Fibrosis
Characterization of a disease-associated mutation affecting a putative splicing regulatory element in intron 6b of the cystic fibrosis transmembrane conductance regulator (CFTR)gene.
Cystic Fibrosis
Characterization of a large human transgene following invasin-mediated delivery in a bacterial artificial chromosome.
Cystic Fibrosis
Characterization of a novel 21-kb deletion, CFTRdele2,3(21 kb), in the CFTR gene: a cystic fibrosis mutation of Slavic origin common in Central and East Europe.
Cystic Fibrosis
Characterization of a recurrent novel large duplication in the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Characterization of an adenovirus gene transfer vector containing an E4 deletion.
Cystic Fibrosis
Characterization of an intron 12 splice donor mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
Characterization of CFTR expression and chloride channel activity in human endothelia.
Cystic Fibrosis
Characterization of CFTR expression in a human pulmonary mucoepidermoid carcinoma cell line, NCI-H292 cells.
Cystic Fibrosis
Characterization of clinical and genetic spectrum of Chinese patients with cystic fibrosis.
Cystic Fibrosis
Characterization of cyclic AMP-regulated chloride conductance in the pigmented rabbit conjunctival epithelial cells.
Cystic Fibrosis
Characterization of Defects in Ion Transport and Tissue Development in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Knockout Rats.
Cystic Fibrosis
Characterization of disease-associated mutations affecting an exonic splicing enhancer and two cryptic splice sites in exon 13 of the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Characterization of factors involved in modulating persistence of transgene expression from recombinant adenovirus in the mouse lung.
Cystic Fibrosis
Characterization of ion channel and transporter mRNA expressions in isolated gill chloride and pavement cells of seawater acclimating eels.
Cystic Fibrosis
Characterization of mitochondrial function in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function.
Cystic Fibrosis
Characterization of more than 85% of cystic fibrosis alleles in the Greek population, including five novel mutations.
Cystic Fibrosis
Characterization of Nasal Potential Difference in cftr Knockout and F508del-CFTR Mice.
Cystic Fibrosis
Characterization of polyclonal and monoclonal antibodies to cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Characterization of the adenosinetriphosphatase and transport activities of purified cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Characterization of the cAMP response element of the cystic fibrosis transmembrane conductance regulator gene promoter.
Cystic Fibrosis
Characterization of the cystic fibrosis transmembrane conductance regulator in a colonocyte cell line.
Cystic Fibrosis
Characterization of the cystic fibrosis transmembrane conductance regulator promoter region. Chromatin context and tissue-specificity.
Cystic Fibrosis
Characterization of the effects of Enterococcus faecium on intestinal epithelial transport properties in piglets.
Cystic Fibrosis
Characterization of the inhibitory effect of boiled rice on intestinal chloride secretion in guinea pig crypt cells.
Cystic Fibrosis
Characterization of the internalization pathways for the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Characterization of the ion transport responses to ADH in the MDCK-C7 cell line.
Cystic Fibrosis
Characterization of the oligosaccharide structures associated with the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Characterization of the promoter region of the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Characterization of the trafficking pathway of cystic fibrosis transmembrane conductance regulator in baby hamster kidney cells.
Cystic Fibrosis
Characterization of two distinct chloride channels in cultured dog pancreatic duct epithelial cells.
Cystic Fibrosis
Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia.
Cystic Fibrosis
Characterizing diverse orthologues of the cystic fibrosis transmembrane conductance regulator protein for structural studies.
Cystic Fibrosis
Characterizing responses to CFTR-modulating drugs using rectal organoids derived from subjects with cystic fibrosis.
Cystic Fibrosis
Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR trafficking.
Cystic Fibrosis
Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein.
Cystic Fibrosis
Chemical chaperones enhance superantigen and conventional antigen presentation by HLA-DM-deficient as well as HLA-DM-sufficient antigen-presenting cells and enhance IgG2a production in vivo.
Cystic Fibrosis
Chemical rescue of deltaF508-CFTR mimics genetic repair in cystic fibrosis bronchial epithelial cells.
Cystic Fibrosis
Chemically modified peptide scaffolds target the CFTR-associated ligand PDZ domain.
Cystic Fibrosis
Chemotoxicity of doxorubicin and surface expression of P-glycoprotein (MDR1) is regulated by the Pseudomonas aeruginosa toxin Cif.
Cystic Fibrosis
Chenodeoxycholic Acid Requires Activation of EGFR, EPAC and Ca2+ to Stimulate CFTR-dependent Cl- Secretion in Human Colonic T84 Cells.
Cystic Fibrosis
Chenodeoxycholic acid stimulates Cl- secretion via cAMP signaling and increases cystic fibrosis transmembrane conductance regulator phosphorylation in T84 cells.
Cystic Fibrosis
Children and adolescents with cystic fibrosis display moderate bone microarchitecture abnormalities: data from high-resolution peripheral quantitative computed tomography.
Cystic Fibrosis
Chimeric constructs endow the human CFTR Cl channel with the gating behavior of murine CFTR.
Cystic Fibrosis
Chitosan Nanocomplexes for the Delivery of ENaC Antisense Oligonucleotides to Airway Epithelial Cells.
Cystic Fibrosis
Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Chloride channel function is linked to epithelium-dependent airway relaxation.
Cystic Fibrosis
Chloride channels in apical membrane of primary cultures of rabbit distal bright convoluted tubule.
Cystic Fibrosis
Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia.
Cystic Fibrosis
Chloride channels in the plasma membrane of a foetal Drosophila cell line, S2.
Cystic Fibrosis
Chloride channels of intracellular organelles and their potential role in cystic fibrosis.
Cystic Fibrosis
Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings.
Cystic Fibrosis
Chloride conductance and sodium-dependent glucose transport in rat and human enterocytes.
Cystic Fibrosis
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
Cystic Fibrosis
Chloride conductance in HT29 cells: investigations with apical membrane vesicles and RT-PCR.
Cystic Fibrosis
Chloride Conductance, Nasal Potential Difference and Cystic Fibrosis Pathophysiology.
Cystic Fibrosis
Chloride currents activated by calcitonin and cAMP in primary cultures of rabbit distal convoluted tubule.
Cystic Fibrosis
Chloride currents in primary cultures of rabbit proximal and distal convoluted tubules.
Cystic Fibrosis
Chloride secretion and function of serous and mucous cells of human airway glands.
Cystic Fibrosis
Chloride secretion in a morphologically differentiated human colonic cell line that expresses the epithelial Na+ channel.
Cystic Fibrosis
Chloride secretion in the trachea of null cystic fibrosis mice: the effects of transfection with pTrial10-CFTR2.
Cystic Fibrosis
Chloride transport in microperfused interlobular ducts isolated from guinea-pig pancreas.
Cystic Fibrosis
Chloride transport-driven alveolar fluid secretion is a major contributor to cardiogenic lung edema.
Cystic Fibrosis
Chloride transporting capability of Calu-3 epithelia following persistent knockdown of the cystic fibrosis transmembrane conductance regulator, CFTR.
Cystic Fibrosis
Chloride turnover and ion-transporting activities of yolk-sac preparations (yolk balls) separated from Mozambique tilapia embryos and incubated in freshwater and seawater.
Cystic Fibrosis
Chloride-dependent secretion of alveolar wall liquid determined by optical-sectioning microscopy.
Cystic Fibrosis
Chlorotoxin does not inhibit volume-regulated, calcium-activated and cyclic AMP-activated chloride channels.
Cystic Fibrosis
Cholesterol Interaction Directly Enhances Intrinsic Activity of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Cystic Fibrosis
Cholesterol modulates cell signaling and protein networking by specifically interacting with PDZ domain-containing scaffold proteins.
Cystic Fibrosis
Cholesterol modulates CFTR confinement in the plasma membrane of primary epithelial cells.
Cystic Fibrosis
Chromanol 293B, a blocker of the slow delayed rectifier K+ current (IKs), inhibits the CFTR Cl- current.
Cystic Fibrosis
Chromatin remodeling mediated by the FOXA1/A2 transcription factors activates CFTR expression in intestinal epithelial cells.
Cystic Fibrosis
Chronic airway infection/inflammation induces a Ca2+i-dependent hyperinflammatory response in human cystic fibrosis airway epithelia.
Cystic Fibrosis
Chronic electronic cigarette exposure in mice induces features of COPD in a nicotine-dependent manner.
Cystic Fibrosis
Chronic inflammation in the cystic fibrosis lung: alterations in inter- and intracellular signaling.
Cystic Fibrosis
Chronic pancreatitis associated with the p.G208A variant of PRSS1 gene in a European patient.
Cystic Fibrosis
Chronic Pancreatitis in the 21st Century - Research Challenges and Opportunities: Summary of a National Institute of Diabetes and Digestive and Kidney Diseases Workshop.
Cystic Fibrosis
Chronic pancreatitis: challenges and advances in pathogenesis, genetics, diagnosis, and therapy.
Cystic Fibrosis
Chronic rhinosinusitis in patients with cystic fibrosis-Current management and new treatments.
Cystic Fibrosis
Cigarette and ENDS preparations differentially regulate ion channels and mucociliary clearance in primary normal human bronchial 3D cultures.
Cystic Fibrosis
Cigarette smoke activates CFTR through ROS-stimulated cAMP signaling in human bronchial epithelial cells.
Cystic Fibrosis
Cigarette Smoke Exposure Induces Retrograde Trafficking of CFTR to the Endoplasmic Reticulum.
Cystic Fibrosis
Cigarette smoke exposure reveals a novel role for the MEK/ERK1/2 MAPK pathway in regulation of CFTR.
Cystic Fibrosis
Cigarette Smoke Induces Systemic Defects in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Function.
Cystic Fibrosis
Cigarette Smoke-Induced Acquired Dysfunction of Cystic Fibrosis Transmembrane Conductance Regulator in the Pathogenesis of Chronic Obstructive Pulmonary Disease.
Cystic Fibrosis
Cigarette Smoke-induced Ca2+ Release Leads to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Dysfunction.
Cystic Fibrosis
Cigarette smoke-induced differential expression of the genes involved in exocrine function of the rat pancreas.
Cystic Fibrosis
cis-Acting elements within CFTR 5'-flanking DNA are not sufficient to decrease gene expression in response to phorbol ester.
Cystic Fibrosis
Cl(-)-dependent HCO3- transport by cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Cl- absorption across the thick ascending limb is not altered in cystic fibrosis mice. A role for a pseudo-CFTR Cl- channel.
Cystic Fibrosis
Cl- channel activity in Xenopus oocytes expressing the cystic fibrosis gene.
Cystic Fibrosis
Cl- channel inhibition by glibenclamide is not specific for the CFTR-type Cl- channel.
Cystic Fibrosis
Cl- channels regulate lipid droplet formation via Rab8a expression during adipocyte differentiation.
Cystic Fibrosis
Cl- is required for HCO3- entry necessary for sperm capacitation in guinea pig: involvement of a Cl-/HCO3- exchanger (SLC26A3) and CFTR.
Cystic Fibrosis
Cl- transport by cystic fibrosis transmembrane conductance regulator (CFTR) contributes to the inhibition of epithelial Na+ channels (ENaCs) in Xenopus oocytes co-expressing CFTR and ENaC.
Cystic Fibrosis
Cl- transport in an immortalized human epithelial cell line (NCM460) derived from the normal transverse colon.
Cystic Fibrosis
Clara cell impact in air-side activation of CFTR in small pulmonary airways.
Cystic Fibrosis
Clarification of laboratory and clinical variables that influence cystic fibrosis newborn screening with initial analysis of immunoreactive trypsinogen.
Cystic Fibrosis
ClC-2 contributes to native chloride secretion by a human intestinal cell line, Caco-2.
Cystic Fibrosis
ClC-3B, a novel ClC-3 splicing variant that interacts with EBP50 and facilitates expression of CFTR-regulated ORCC.
Cystic Fibrosis
ClC-5: ontogeny of an alternative chloride channel in respiratory epithelia.
Cystic Fibrosis
CLCA4 variants determine the manifestation of the cystic fibrosis basic defect in the intestine.
Cystic Fibrosis
Clinical and genetic features of patients with congenital unilateral absence of the vas deferens.
Cystic Fibrosis
Clinical and genetic risk factors for cystic fibrosis-related liver disease.
Cystic Fibrosis
Clinical and radiological outcome of patients suffering from chronic pancreatitis associated with gene mutations.
Cystic Fibrosis
Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model.
Cystic Fibrosis
Clinical Effectiveness of Lumacaftor/Ivacaftor in Cystic Fibrosis Patients Homozygous for F508del-CFTR.
Cystic Fibrosis
Clinical evaluation of the infertile male with respect to genetic etiologies.
Cystic Fibrosis
Clinical evaluation of the Nanoduct sweat test system in the diagnosis of cystic fibrosis after newborn screening.
Cystic Fibrosis
Clinical Features and Endoscopic Treatment of Chinese Patients With Hereditary Pancreatitis.
Cystic Fibrosis
Clinical genetic testing for male factor infertility: current applications and future directions.
Cystic Fibrosis
Clinical hallmarks and genetic polymorphisms in the CFTR gene contribute to the disclosure of the A1006E mutation.
Cystic Fibrosis
Clinical implications of cystic fibrosis transmembrane conductance regulator mutations.
Cystic Fibrosis
Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.
Cystic Fibrosis
Clinical outcomes in infants with cystic fibrosis transmembrane conductance regulator (CFTR) related metabolic syndrome.
Cystic Fibrosis
Clinical phenotype and genotype of children with borderline sweat test and abnormal nasal epithelial chloride transport.
Cystic Fibrosis
Clinical practice and genetic counseling for cystic fibrosis and CFTR-related disorders.
Cystic Fibrosis
Clinically Applicable Procedure for Gene Delivery to Fetal Gut by Ultrasound-Guided Gastric Injection: Toward Prenatal Prevention of Early-Onset Intestinal Diseases.
Cystic Fibrosis
Clinically applicable procedure for gene delivery to fetal gut by ultrasound-guided gastric injection: toward prenatal prevention of early-onset intestinal diseases.
Cystic Fibrosis
Clinically-approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia.
Cystic Fibrosis
Cloning and sequence analysis of rat cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Cloning the mouse homolog of the human cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Cloning, characterization, and functional expression of a CNP receptor regulating CFTR in the shark rectal gland.
Cystic Fibrosis
Cloning, functional expression, and characterization of the human prostaglandin E2 receptor EP2 subtype.
Cystic Fibrosis
Clusters of Cl- channels in CFTR-expressing Sf9 cells switch spontaneously between slow and fast gating modes.
Cystic Fibrosis
Co- and posttranslational translocation mechanisms direct cystic fibrosis transmembrane conductance regulator N terminus transmembrane assembly.
Cystic Fibrosis
Cocoa-related flavonoids inhibit CFTR-mediated chloride transport across T84 human colon epithelia.
Cystic Fibrosis
Coculture of Staphylococcus aureus with Pseudomonas aeruginosa Drives S. aureus towards Fermentative Metabolism and Reduced Viability in a Cystic Fibrosis Model.
Cystic Fibrosis
Codon bias and the folding dynamics of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Combination of Correctors Rescue ?F508-CFTR by Reducing Its Association with Hsp40 and Hsp27.
Cystic Fibrosis
Combination of Correctors Rescues CFTR Transmembrane-Domain Mutants by Mitigating their Interactions with Proteostasis.
Cystic Fibrosis
Combination Therapy with Cystic Fibrosis Transmembrane Conductance Regulator Modulators Augment the Airway Functional Microanatomy.
Cystic Fibrosis
Combinatorial effects of genistein and sex-steroids on the level of cystic fibrosis transmembrane regulator (CFTR), adenylate cyclase (AC) and cAMP in the cervix of ovariectomised rats.
Cystic Fibrosis
Combined Computational-Experimental Analyses of CFTR Exon Strength Uncover Predictability of Exon Skipping Level.
Cystic Fibrosis
Combined Use of CFTR Correctors in LGMD2D Myotubes Improves Sarcoglycan Complex Recovery.
Cystic Fibrosis
Combining theoretical and experimental data to decipher CFTR 3D structures and functions.
Cystic Fibrosis
Commensal bacteria increase invasion of intestinal epithelium by Salmonella enterica serovar Typhi.
Cystic Fibrosis
Commentary for the article: MicroRNA-1246 regulates proliferation, invasion and differentiation in human vascular smooth muscle cell by targeting cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
Common CFTR gene variants influence body composition and survival in rural Ghana.
Cystic Fibrosis
Common CFTR mutations are not likely to predispose to chronic bronchitis in northern Germany.
Cystic Fibrosis
Compacted DNA nanoparticles administered to the nasal mucosa of cystic fibrosis subjects are safe and demonstrate partial to complete cystic fibrosis transmembrane regulator reconstitution.
Cystic Fibrosis
Comparative analysis of common CFTR polymorphisms poly-T, TG-repeats and M470V in a healthy Chinese population.
Cystic Fibrosis
Comparative genomic sequence analysis of the human and mouse cystic fibrosis transmembrane conductance regulator genes.
Cystic Fibrosis
Comparative pharmacology of the activity of wild-type and G551D mutated CFTR chloride channel: effect of the benzimidazolone derivative NS004.
Cystic Fibrosis
Comparative processing and function of human and ferret cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Comparison of Ambient and Atmospheric Pressure Ion Sources for Cystic Fibrosis Exhaled Breath Condensate Ion Mobility-Mass Spectrometry Metabolomics.
Cystic Fibrosis
Comparison of Cas9 and Cas12a CRISPR editing methods to correct the W1282X-CFTR mutation.
Cystic Fibrosis
Comparison of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) and Ciliary Beat Frequency Activation by the CFTR Modulators Genistein, VRT-532, and UCCF-152 in Primary Sinonasal Epithelial Cultures.
Cystic Fibrosis
Comparison of Nasal Potential Difference and Intestinal Current Measurements as Surrogate Markers for CFTR function.
Cystic Fibrosis
Comparison of radiographic and clinical characteristics of low-risk and high-risk cystic fibrosis genotypes.
Cystic Fibrosis
Comparison of the bacterial HelA protein to the F508 region of the cystic fibrosis transmembrane regulator.
Cystic Fibrosis
Comparison of the gating behaviour of human and murine cystic fibrosis transmembrane conductance regulator Cl- channels expressed in mammalian cells.
Cystic Fibrosis
Comparison of two different protocols of neonatal screening for cystic fibrosis.
Cystic Fibrosis
Comparison of vectorial ion transport in primary murine airway and human sinonasal air-liquid interface cultures, models for studies of cystic fibrosis, and other airway diseases.
Cystic Fibrosis
Comparisons of two types of teleostean pseudobranchs, silver moony (Monodactylus argenteus) and tilapia (Oreochromis mossambicus), with salinity-dependent morphology and ion transporter expression.
Cystic Fibrosis
Compartmentalized Accumulation of cAMP near complexes of Multidrug Resistance Protein 4 (MRP4) and Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Contributes to Drug-induced Diarrhea.
Cystic Fibrosis
Compartmentalized autocrine signaling to cystic fibrosis transmembrane conductance regulator at the apical membrane of airway epithelial cells.
Cystic Fibrosis
Compartmentalized cyclic adenosine 3',5'-monophosphate at the plasma membrane clusters PDE3A and cystic fibrosis transmembrane conductance regulator into microdomains.
Cystic Fibrosis
Complement yourself: Transcomplementation rescues partially folded mutant proteins.
Cystic Fibrosis
Complete and rapid scanning of the cystic fibrosis transmembrane conductance regulator (CFTR) gene by denaturing high-performance liquid chromatography (D-HPLC): major implications for genetic counselling.
Cystic Fibrosis
Complete ascertainment of intragenic copy number mutations (CNMs) in the CFTR gene and its implications for CNM formation at other autosomal loci.
Cystic Fibrosis
Complete cystic fibrosis transmembrane conductance regulator gene sequencing in patients with idiopathic chronic pancreatitis and controls.
Cystic Fibrosis
Complete gene scanning by temperature gradient capillary electrophoresis using the cystic fibrosis transmembrane conductance regulator gene as a model.
Cystic Fibrosis
Complete identification of cystic fibrosis transmembrane conductance regulator mutations in the CF population of Saguenay Lac-Saint-Jean (Quebec, Canada).
Cystic Fibrosis
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
Cystic Fibrosis
Complete mutational screening of the cystic fibrosis transmembrane conductance regulator gene: cystic fibrosis mutations are not involved in healthy men with reduced sperm quality.
Cystic Fibrosis
Complex allele [-102T>A+S549R(T>G)] is associated with milder forms of cystic fibrosis than allele S549R(T>G) alone.
Cystic Fibrosis
Complexity of phenotypes induced by p.Asn1303Lys-CFTR correlates with difficulty to rescue and activate this protein.
Cystic Fibrosis
Composition of mineralizing incisor enamel in cystic fibrosis transmembrane conductance regulator-deficient mice.
Cystic Fibrosis
Compound genetic abnormalities in patients with cystic fibrosis transmembrane regulator gene mutation.
Cystic Fibrosis
Compound heterozygosity for the delta F508 and F508C cystic fibrosis transmembrane conductance regulator (CFTR) mutations in a patient with congenital bilateral aplasia of the vas deferens.
Cystic Fibrosis
Comprehensive and accurate mutation scanning of the CFTR gene by two-dimensional DNA electrophoresis.
Cystic Fibrosis
Comprehensive and Rapid Genotyping of Mutations and Haplotypes in Congenital Bilateral Absence of the Vas Deferens and Other Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders.
Cystic Fibrosis
Comprehensive cystic fibrosis mutation epidemiology and haplotype characterization in a southern Italian population.
Cystic Fibrosis
Comprehensive genetic analysis of the cystic fibrosis transmembrane conductance regulator from dried blood specimens - Implications for newborn screening.
Cystic Fibrosis
Comprehensive screening for PRSS1, SPINK1, CFTR, CTRC and CLDN2 gene mutations in Chinese paediatric patients with idiopathic chronic pancreatitis: a cohort study.
Cystic Fibrosis
Computational Analysis of Energy Landscapes Reveals Dynamic Features That Contribute to Binding of Inhibitors to CFTR-Associated Ligand.
Cystic Fibrosis
Computational design of a PDZ domain peptide inhibitor that rescues CFTR activity.
Cystic Fibrosis
Computational studies reveal phosphorylation-dependent changes in the unstructured R domain of CFTR.
Cystic Fibrosis
Computed Tomography (CT) Scanning Facilitates Early Identification of Neonatal Cystic Fibrosis Piglets.
Cystic Fibrosis
Computed tomography correlates with improvement with ivacaftor in cystic fibrosis patients with G551D mutation.
Cystic Fibrosis
Concentration of fractional excretion of nitric oxide (FENO): A potential airway biomarker of restored CFTR function.
Cystic Fibrosis
Confirming the phylogeny of mammals by use of large comparative sequence data sets.
Cystic Fibrosis
Conformation, independent of charge, in the R domain affects cystic fibrosis transmembrane conductance regulator channel openings.
Cystic Fibrosis
Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments.
Cystic Fibrosis
Conformational change of the extracellular parts of the CFTR protein during channel gating.
Cystic Fibrosis
Conformational change opening the CFTR chloride channel pore coupled to ATP-dependent gating.
Cystic Fibrosis
Conformational changes in a pore-lining helix coupled to cystic fibrosis transmembrane conductance regulator channel gating.
Cystic Fibrosis
Conformational changes in the catalytically inactive nucleotide-binding site of CFTR.
Cystic Fibrosis
Conformational Changes Relevant to Channel Activity and Folding within the first Nucleotide Binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
Congenital absence of the vas deferens: incomplete penetrance of cystic fibrosis gene mutations.
Cystic Fibrosis
Congenital bilateral absence of the vas deferens (CBAVD) and cystic fibrosis transmembrane regulator (CFTR): correlation between genotype and phenotype.
Cystic Fibrosis
Congenital bilateral absence of the vas deferens (CBAVD): do genetic disorders modify assisted reproductive technologies outcomes?
Cystic Fibrosis
Congenital bilateral absence of the vas deferens as an atypical form of cystic fibrosis: reproductive implications and genetic counseling.
Cystic Fibrosis
Congenital bilateral absence of the vas deferens: clinical characteristics, biological parameters, cystic fibrosis transmembrane conductance regulator gene mutations, and implications for genetic counseling.
Cystic Fibrosis
Congenital bilateral absence of vas deferens with a new missense mutation (P499A) in the CFTR gene.
Cystic Fibrosis
Congenital Chloride-losing Diarrhea Causing Mutations in the STAS Domain Result in Misfolding and Mistrafficking of SLC26A3.
Cystic Fibrosis
Connections Between Genetics and Clinical Data: Role of MCP-1, CFTR, and SPINK-1 in the Setting of Acute, Acute Recurrent, and Chronic Pancreatitis.
Cystic Fibrosis
Connectivity mapping (ssCMap) to predict A20-inducing drugs and their antiinflammatory action in cystic fibrosis.
Cystic Fibrosis
Conservation of CFTR codon frequency through primates suggests synonymous mutations could have a functional effect.
Cystic Fibrosis
Conserved allosteric hot spots in the transmembrane domains of cystic fibrosis transmembrane conductance regulator (CFTR) channels and multidrug resistance protein (MRP) pumps.
Cystic Fibrosis
Considerations for the Conduct of Clinical Trials with Antiinflammatory Agents in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report.
Cystic Fibrosis
Constitutive activation of nuclear factor ?B contributes to cystic fibrosis transmembrane conductance regulator expression and promotes human cervical cancer progression and poor prognosis.
Cystic Fibrosis
Constitutive internalization of cystic fibrosis transmembrane conductance regulator occurs via clathrin-dependent endocytosis and is regulated by protein phosphorylation.
Cystic Fibrosis
Constrained bithiazoles: small molecule correctors of defective ?F508-CFTR protein trafficking.
Cystic Fibrosis
Contractile Properties of Intrapulmonary Airway Smooth Muscle in Cystic Fibrosis.
Cystic Fibrosis
Contribution of a leucine residue in the first transmembrane segment to the selectivity filter region in the CFTR chloride channel.
Cystic Fibrosis
Contribution of CFTR to alveolar fluid clearance by lipoxin A4 via PI3K/Akt pathway in LPS-induced acute lung injury.
Cystic Fibrosis
Contribution of concentration-sensitive sodium channels to the absorption of alveolar fluid in mice.
Cystic Fibrosis
Contribution of M470V variant to cystic fibrosis: First study in CF and normal Tunisian population.
Cystic Fibrosis
Contribution of proline residues in the membrane-spanning domains of cystic fibrosis transmembrane conductance regulator to chloride channel function.
Cystic Fibrosis
Contribution of R domain phosphoserines to the function of CFTR studied in Fischer rat thyroid epithelia.
Cystic Fibrosis
Contribution of the CFTR gene, the pancreatic secretory trypsin inhibitor gene (SPINK1) and the cationic trypsinogen gene (PRSS1) to the etiology of recurrent pancreatitis.
Cystic Fibrosis
Contribution of the eighth transmembrane segment to the function of the CFTR chloride channel pore.
Cystic Fibrosis
Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis.
Cystic Fibrosis
Control of CFTR chloride conductance by ATP levels through non-hydrolytic binding.
Cystic Fibrosis
Control of cystic fibrosis transmembrane conductance regulator expression by BAP31.
Cystic Fibrosis
Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi.
Cystic Fibrosis
Control of dynamic CFTR selectivity by glutamate and ATP in epithelial cells.
Cystic Fibrosis
Control of epithelial Na+ conductance by the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Control of the cystic fibrosis transmembrane conductance regulator by alphaG(i) and RGS proteins.
Cystic Fibrosis
Convective washout reduces the antidiarrheal efficacy of enterocyte surface-targeted antisecretory drugs.
Cystic Fibrosis
Convergence of IRBIT, phosphatidylinositol (4,5) bisphosphate, and WNK/SPAK kinases in regulation of the Na+-HCO3- cotransporters family.
Cystic Fibrosis
Converting nonhydrolyzable nucleotides to strong cystic fibrosis transmembrane conductance regulator (CFTR) agonists by gain of function (GOF) mutations.
Cystic Fibrosis
COOH-terminal truncations promote proteasome-dependent degradation of mature cystic fibrosis transmembrane conductance regulator from post-Golgi compartments.
Cystic Fibrosis
Cooperativity and flexibility of cystic fibrosis transmembrane conductance regulator transmembrane segments participate in membrane localization of a charged residue.
Cystic Fibrosis
Coordinate upregulation of guanylin and uroguanylin expression by hypertonicity in HT29-18-N2 cells.
Cystic Fibrosis
Coordinated release of nucleotides and mucin from human airway epithelial Calu-3 cells.
Cystic Fibrosis
Coordination of pancreatic HCO3- secretion by protein-protein interaction between membrane transporters.
Cystic Fibrosis
COPII machinery cooperates with ER-localized Hsp40 to sequester misfolded membrane proteins into ER-associated compartments.
Cystic Fibrosis
COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code.
Cystic Fibrosis
Copy number-dependent expression of a YAC-cloned human CFTR gene in a human epithelial cell line.
Cystic Fibrosis
CORK Study in Cystic Fibrosis: Sustained Improvements in Ultra-Low-Dose Chest CT Scores After CFTR Modulation With Ivacaftor.
Cystic Fibrosis
Coronavirus 3CLpro proteinase cleavage sites: possible relevance to SARS virus pathology.
Cystic Fibrosis
Correcting CFTR: New Gene Editing Strategies for Rescuing CFTR Function Ex Vivo.
Cystic Fibrosis
Correcting the F508del-CFTR variant by modulating eukaryotic translation initiation factor 3-mediated translation initiation.
Cystic Fibrosis
Correction of aberrant splicing of the cystic fibrosis transmembrane conductance regulator (CFTR) gene by antisense oligonucleotides.
Cystic Fibrosis
Correction of Airway Stem Cells: Genome Editing Approaches for the Treatment of Cystic Fibrosis.
Cystic Fibrosis
Correction of cAMP-stimulated fluid secretion in cystic fibrosis airway epithelia: efficiency of adenovirus-mediated gene transfer in vitro.
Cystic Fibrosis
Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators.
Cystic Fibrosis
Correction of CFTR malfunction and stimulation of Ca-activated Cl channels restore HCO3- secretion in cystic fibrosis bile ductular cells.
Cystic Fibrosis
Correction of defective protein kinesis of human P-glycoprotein mutants by substrates and modulators.
Cystic Fibrosis
Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells.
Cystic Fibrosis
Correction of F508del-CFTR trafficking by the sponge alkaloid latonduine is modulated by interaction with PARP.
Cystic Fibrosis
Correction of G551D-CFTR transport defect in epithelial monolayers by genistein but not by CPX or MPB-07.
Cystic Fibrosis
Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR.
Cystic Fibrosis
Correction of mutations within the cystic fibrosis transmembrane conductance regulator by site-directed RNA editing.
Cystic Fibrosis
Correction of the ?F508 Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator Gene by Zinc-Finger Nuclease Homology-Directed Repair.
Cystic Fibrosis
Correction of the apical membrane chloride permeability defect in polarized cystic fibrosis airway epithelia following retroviral-mediated gene transfer.
Cystic Fibrosis
Correction of the cystic fibrosis defect by gene complementation in human intrahepatic biliary epithelial cell lines.
Cystic Fibrosis
Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer.
Cystic Fibrosis
Correction of the ion transport defect in cystic fibrosis transgenic mice by gene therapy.
Cystic Fibrosis
Correction to Molecular Basis for Fe(III)-Independent Curcumin Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Activity.
Cystic Fibrosis
Correction: cAMP/Protein Kinase A Activates Cystic Fibrosis Transmembrane Conductance Regulator for ATP Release from Rat Skeletal Muscle during Low pH or Contractions.
Cystic Fibrosis
Correction: Learning from each other: ABC transporter regulation by protein phosphorylation in plant and mammalian systems.
Cystic Fibrosis
Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del).
Cystic Fibrosis
Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis.
Cystic Fibrosis
Correctors and Potentiators Rescue Function of the Truncated W1282X-Cystic Fibrosis Transmembrane Regulator (CFTR) Translation Product.
Cystic Fibrosis
Correctors enhance maturation of DeltaF508 CFTR by promoting interactions between the two halves of the molecule.
Cystic Fibrosis
Correctors promote maturation of cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by binding to the protein.
Cystic Fibrosis
Correctors Rescue CFTR Mutations in Nucleotide-Binding Domain 1 (NBD1) by Modulating Proteostasis.
Cystic Fibrosis
Correlating Cystic Fibrosis Transmembrane Conductance Regulator Function with Clinical Features to Inform Precision Treatment of Cystic Fibrosis.
Cystic Fibrosis
Correlation between genotype and phenotype in patients with cystic fibrosis. The Cystic Fibrosis Genotype-Phenotype Consortium.
Cystic Fibrosis
Correlation between nasal potential difference measurements, genotype and clinical condition in patients with cystic fibrosis.
Cystic Fibrosis
Correlation of apical fluid-regulating channel proteins with lung function in human COPD lungs.
Cystic Fibrosis
Correlation of sweat chloride concentration with classes of the cystic fibrosis transmembrane conductance regulator gene mutations.
Cystic Fibrosis
Correlation of sweat chloride concentration with genotypes in cystic fibrosis patients in Saguenay Lac-Saint-Jean, Quebec, Canada.
Cystic Fibrosis
Correlations between cystic fibrosis genotype and sinus disease severity in chronic rhinosinusitis.
Cystic Fibrosis
Corrigendum to "Testosterone regulates levels of cystic fibrosis transmembrane regulator, adenylate cyclase and cAMP in the seminal vesicles of orchidectomized rats" [Theriogenology 85(2) (2016) 238-246].
Cystic Fibrosis
Corticosteroid use and increased CXCR2 levels on leukocytes are associated with lumacaftor/ivacaftor discontinuation in cystic fibrosis patients homozygous for the F508del CFTR mutation.
Cystic Fibrosis
Corticosteroids correct aberrant CFTR localization in the duct and regenerate acinar cells in autoimmune pancreatitis.
Cystic Fibrosis
Cortisol regulates insulin-like growth-factor binding protein (igfbp) gene expression in Atlantic salmon parr.
Cystic Fibrosis
Cortisol regulation of ion transporter mRNA in Atlantic salmon gill and the effect of salinity on the signaling pathway.
Cystic Fibrosis
Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro.
Cystic Fibrosis
Could growth retardation in cystic fibrosis be partly due to deficient steroid and thyroid hormonogenesis?
Cystic Fibrosis
Counteracting suppression of CFTR and voltage-gated K+ channels by a bacterial pathogenic factor with the natural product tannic acid.
Cystic Fibrosis
Coupled movement of permeant and blocking ions in the CFTR chloride channel pore.
Cystic Fibrosis
Coupled secretion of chloride and mucus in skin of Xenopus laevis: possible role for CFTR.
Cystic Fibrosis
Coupling cystic fibrosis to endoplasmic reticulum stress: Differential role of Grp78 and ATF6.
Cystic Fibrosis
Coupling of ATP hydrolysis with channel gating by purified, reconstituted CFTR.
Cystic Fibrosis
Covalent modification of the nucleotide binding domains of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Covalent modification of the regulatory domain irreversibly stimulates cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
COX2 in a euryhaline teleost, Fundulus heteroclitus: primary sequence, distribution, localization, and potential function in gills during salinity acclimation.
Cystic Fibrosis
CpABC, a Cryptosporidium parvum ATP-binding cassette protein at the host-parasite boundary in intracellular stages.
Cystic Fibrosis
CPX, a selective A1-adenosine-receptor antagonist, regulates intracellular pH in cystic fibrosis cells.
Cystic Fibrosis
Critical modifier role of membrane-cystic fibrosis transmembrane conductance regulator-dependent ceramide signaling in lung injury and emphysema.
Cystic Fibrosis
Critical role of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm.
Cystic Fibrosis
Crofelemer for the treatment of chronic diarrhea in patients living with HIV/AIDS.
Cystic Fibrosis
Crofelemer, a novel antisecretory agent approved for the treatment of HIV-associated diarrhea.
Cystic Fibrosis
CROFELEMER, AN ANTI-SECRETORY ANTIDIARRHEAL PROANTHOCYANIDIN OLIGOMER EXTRACTED FROM CROTON LECHLERI, TARGETS TWO DISTINCT INTESTINAL CHLORIDE CHANNELS.
Cystic Fibrosis
Crofelemer: In HIV Associated Diarrhea and Secretory Diarrhea - A Patent Perspective.
Cystic Fibrosis
Cross talk of cAMP and flavone in regulation of cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel and Na+/K+/2Cl- cotransporter in renal epithelial A6 cells.
Cystic Fibrosis
Cross-species characterization of the promoter region of the cystic fibrosis transmembrane conductance regulator gene reveals multiple levels of regulation.
Cystic Fibrosis
Crucial role for phylogenetically conserved cytoplasmic loop 3 in ABCC4 protein expression.
Cystic Fibrosis
Cryo-EM Visualization of an Active High Open Probability CFTR Anion Channel.
Cystic Fibrosis
Cryptorchidism-induced CFTR down-regulation results in disruption of testicular tight junctions through up-regulation of NF-?B/COX-2/PGE2.
Cystic Fibrosis
Crystal Structure of Isoform CBd of the Basic Phospholipase A2 Subunit of Crotoxin: Description of the Structural Framework of CB for Interaction with Protein Targets.
Cystic Fibrosis
Crystal structure of the cystic fibrosis transmembrane conductance regulator inhibitory factor Cif reveals novel active-site features of an epoxide hydrolase virulence factor.
Cystic Fibrosis
Crystal structure of the PDZ1 domain of human Na(+)/H(+) exchanger regulatory factor provides insights into the mechanism of carboxyl-terminal leucine recognition by class I PDZ domains.
Cystic Fibrosis
Crystallographic and single-particle analyses of native- and nucleotide-bound forms of the cystic fibrosis transmembrane conductance regulator (CFTR) protein.
Cystic Fibrosis
Crystallographic characterization of the PDZ1 domain of the human Na+/H+ exchanger regulatory factor.
Cystic Fibrosis
CTCF regulates positioning of the human cystic fibrosis gene in association with a histone deacetylase.
Cystic Fibrosis
Culture-dependent expression of Na+ conductances in airway epithelial cells.
Cystic Fibrosis
Curcumin cross-links cystic fibrosis transmembrane conductance regulator (CFTR) polypeptides and potentiates CFTR channel activity by distinct mechanisms.
Cystic Fibrosis
Curcumin enhances cystic fibrosis transmembrane regulator expression by down-regulating calreticulin.
Cystic Fibrosis
Curcumin opens cystic fibrosis transmembrane conductance regulator channels by a novel mechanism that requires neither ATP binding nor dimerization of the nucleotide-binding domains.
Cystic Fibrosis
Curcumin stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.
Cystic Fibrosis
Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects.
Cystic Fibrosis
Current and novel therapeutic strategies for the management of cystic fibrosis.
Cystic Fibrosis
Current concepts: host-pathogen interactions in cystic fibrosis airways disease.
Cystic Fibrosis
Current insights into the role of PKA phosphorylation in CFTR channel activity and the pharmacological rescue of cystic fibrosis disease-causing mutants.
Cystic Fibrosis
Current status and future directions of gene and cell therapy for cystic fibrosis.
Cystic Fibrosis
Current Status of Genetic Diagnosis Laboratories and Frequency of Genetic Variants Associated with Cystic Fibrosis through a Newborn-Screening Program in Turkey.
Cystic Fibrosis
Cyanoquinolines with Independent Corrector and Potentiator Activities Restore {triangleup}F508-CFTR Chloride Channel Function in Cystic Fibrosis.
Cystic Fibrosis
Cyclic AMP regulates bicarbonate secretion in cholangiocytes through release of ATP into bile.
Cystic Fibrosis
Cyclic AMP-dependent protein kinase activation of cystic fibrosis transmembrane conductance regulator chloride channels in planar lipid bilayers.
Cystic Fibrosis
Cyclic AMP-induced K+ secretion occurs independently of Cl- secretion in rat distal colon.
Cystic Fibrosis
Cyclic Peptidyl Inhibitors against CAL/CFTR Interaction for Treatment of Cystic Fibrosis.
Cystic Fibrosis
Cyst growth in ADPKD is prevented by pharmacological and genetic inhibition of TMEM16A in vivo.
Cystic Fibrosis
Cysteamine re-establishes the clearance of Pseudomonas aeruginosa by macrophages bearing the cystic fibrosis-relevant F508del-CFTR mutation.
Cystic Fibrosis
Cysteine accessibility probes timing and extent of NBD separation along the dimer interface in gating CFTR channels.
Cystic Fibrosis
Cysteine modification reveals an allosteric inhibitory site on the CAL PDZ domain.
Cystic Fibrosis
Cysteine residues in the nucleotide binding domains regulate the conductance state of CFTR channels.
Cystic Fibrosis
Cysteine Scanning of CFTR's First Transmembrane Segment Reveals Its Plausible Roles in Gating and Permeation.
Cystic Fibrosis
Cysteine string protein interacts with and modulates the maturation of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis.
Cystic Fibrosis
Cysteine string protein promotes proteasomal degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) by increasing its interaction with the C terminus of Hsp70-interacting protein and promoting CFTR ubiquitylation.
Cystic Fibrosis
Cysteine substitutions reveal dual functions of the amino-terminal tail in cystic fibrosis transmembrane conductance regulator channel gating.
Cystic Fibrosis
Cysteine-independent inhibition of the CFTR chloride channel by the cysteine-reactive reagent sodium (2-sulphonatoethyl) methanethiosulphonate.
Cystic Fibrosis
Cysteine-scanning mutagenesis provides no evidence for the extracellular accessibility of the nucleotide-binding domains of the multidrug resistance transporter P-glycoprotein.
Cystic Fibrosis
Cystic fibrosis ?f508 mutation screening in Brazilian women with altered fertility.
Cystic Fibrosis
Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.
Cystic Fibrosis
Cystic fibrosis airway epithelium remodelling: involvement of inflammation.
Cystic Fibrosis
Cystic fibrosis and bone disease: defective osteoblast maturation with the f508del mutation in cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Cystic fibrosis and Chiari type I malformation: autopsy study of two infants with a rare association.
Cystic Fibrosis
Cystic fibrosis and infertility caused by congenital bilateral absence of the vas deferens and related clinical entities.
Cystic Fibrosis
Cystic fibrosis and innate immunity: how chloride channel mutations provoke lung disease.
Cystic Fibrosis
Cystic Fibrosis and the Cells of the Airway Epithelium: What Are Ionocytes and What Do They Do?
Cystic Fibrosis
Cystic fibrosis as a disease of misprocessing of the cystic fibrosis transmembrane conductance regulator glycoprotein.
Cystic Fibrosis
Cystic fibrosis conductance regulator, tumor necrosis factor, interferon alpha-10, interferon alpha-17, and interferon gamma genotyping as potential risk markers in pulmonary sarcoidosis pathogenesis in Greek patients.
Cystic Fibrosis
Cystic fibrosis drug ivacaftor stimulates CFTR channels at picomolar concentrations.
Cystic Fibrosis
Cystic fibrosis epithelial cells are primed for apoptosis as a result of increased Fas (CD95).
Cystic Fibrosis
Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells.
Cystic Fibrosis
Cystic Fibrosis Foundation Pulmonary Guidelines. Use of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy in Patients with Cystic Fibrosis.
Cystic Fibrosis
Cystic Fibrosis Gene Mutation Frequency Among a Group of Suspected Children in King Hussein Medical Center.
Cystic Fibrosis
Cystic fibrosis gene mutation reduces epithelial cell acidification and injury in acid-perfused mouse duodenum.
Cystic Fibrosis
Cystic fibrosis gene mutations and infertile men with primary testicular failure.
Cystic Fibrosis
Cystic fibrosis gene mutations and polymorphisms in Saudi men with infertility.
Cystic Fibrosis
Cystic fibrosis gene mutations deltaF508 and 394delTT in patients with chronic sinusitis in Finland.
Cystic Fibrosis
Cystic fibrosis genotype and assessing rates of decline in pulmonary status.
Cystic Fibrosis
Cystic fibrosis hetero- and homozygosity is associated with inhibition of breast cancer growth.
Cystic Fibrosis
Cystic fibrosis in a boy with meconium ileus and mild clinical phenotype associated with 2183AA-G/D1152H genotype.
Cystic Fibrosis
Cystic fibrosis in Korean children:a case report identified by a quantitative pilocarpine iontophoresis sweat test and genetic analysis.
Cystic Fibrosis
Cystic fibrosis in South Africa: spectrum of disease and determinants of outcome.
Cystic Fibrosis
Cystic fibrosis in the kidney: new lessons from impaired renal HCO3- excretion.
Cystic Fibrosis
Cystic fibrosis infant care challenges in diagnosis and management in the era of newborn screening.
Cystic Fibrosis
Cystic fibrosis is associated with a defect in apical receptor-mediated endocytosis in mouse and human kidney.
Cystic Fibrosis
Cystic Fibrosis Lung Disease Modifiers and Their Relevance in the New Era of Precision Medicine.
Cystic Fibrosis
Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations.
Cystic Fibrosis
Cystic fibrosis mouse model-dependent intestinal structure and gut microbiome.
Cystic Fibrosis
Cystic fibrosis mRNA expression in rat brain: cerebral cortex and medial preoptic area.
Cystic Fibrosis
Cystic fibrosis mutation classes in pediatric otitis media - Fickle or faulty?
Cystic Fibrosis
Cystic fibrosis mutation detection by hybridization to light-generated DNA probe arrays.
Cystic Fibrosis
Cystic fibrosis mutation screening in CBAVD patients and men with obstructive azoospermia or severe oligozoospermia.
Cystic Fibrosis
Cystic fibrosis mutation screening in healthy men with reduced sperm quality.
Cystic Fibrosis
Cystic fibrosis mutations impair the fertilization rate of epididymal sperm from men with congenital absence of the vas deferens.
Cystic Fibrosis
Cystic fibrosis mutations in French Canadians: three CFTR mutations are relatively frequent in a Quebec population with an elevated incidence of cystic fibrosis.
Cystic Fibrosis
Cystic fibrosis mutations lead to carboxyl-terminal fragments that highlight an early biogenesis step of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Cystic Fibrosis Mutations with Widely Variable Phenotype: The D1152H Example.
Cystic Fibrosis
Cystic fibrosis neutrophils have normal intrinsic reactive oxygen species generation.
Cystic Fibrosis
Cystic fibrosis newborn screening: the importance of bloodspot sample quality.
Cystic Fibrosis
Cystic fibrosis of the pancreas: involvement of MUC6 mucin in obstruction of pancreatic ducts.
Cystic Fibrosis
Cystic Fibrosis of the Pancreas: The Role of CFTR Channel in the Regulation of Intracellular Ca2+ Signaling and Mitochondrial Function in the Exocrine Pancreas.
Cystic Fibrosis
Cystic fibrosis pathogens activate Ca2+-dependent mitogen-activated protein kinase signaling pathways in airway epithelial cells.
Cystic Fibrosis
Cystic fibrosis patients bearing both the common missense mutation Gly----Asp at codon 551 and the delta F508 mutation are clinically indistinguishable from delta F508 homozygotes, except for decreased risk of meconium ileus.
Cystic Fibrosis
Cystic fibrosis patients of minority race and ethnicity less likely eligible for CFTR modulators based on CFTR genotype.
Cystic Fibrosis
Cystic fibrosis patients with the 3272-26A-->G mutation have mild disease, leaky alternative mRNA splicing, and CFTR protein at the cell membrane.
Cystic Fibrosis
Cystic fibrosis phenotype associated with pancreatic insufficiency does not always reflect the cAMP-dependent chloride conductive pathway defect. Analysis of C225R-CFTR and R1066C-CFTR.
Cystic Fibrosis
Cystic fibrosis phenotype evaluation and paternity outcome in 50 males with congenital bilateral absence of vas deferens.
Cystic Fibrosis
Cystic fibrosis presentation in del. F508 and p. Tyr109Glyfs compound heterozygote CFTR state: a case report.
Cystic Fibrosis
Cystic fibrosis presenting as acute pancreatitis and obstructive azoospermia in a young adult male with a novel mutation in the CFTR gene.
Cystic Fibrosis
Cystic Fibrosis Presenting as Pseudo-Bartter Syndrome: An Important Diagnosis that is Missed!
Cystic Fibrosis
Cystic fibrosis presenting as recurrent pancreatitis in a young child with a normal sweat test and pancreas divisum: a case report.
Cystic Fibrosis
Cystic fibrosis presenting with hypokalemia and metabolic alkalosis in a previously healthy adolescent.
Cystic Fibrosis
Cystic fibrosis presenting with persistent pneumonia --a case study of a 3-month-old adopted baby.
Cystic Fibrosis
Cystic fibrosis respiratory tract salt concentration: An Exploratory Cohort Study.
Cystic Fibrosis
Cystic fibrosis swine fail to secrete airway surface liquid in response to inhalation of pathogens.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) activity in nasal epithelial cells from cystic fibrosis patients with severe genotypes.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) allelic variants relate to shifts in faecal microbiota of cystic fibrosis patients.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) and autophagy: hereditary defects in cystic fibrosis versus gluten-mediated inhibition in celiac disease.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) and renal function.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) anion binding as a probe of the pore.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel and Na-K-Cl cotransporter NKCC1 isoform mediate the vasorelaxant action of genistein in isolated rat aorta.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) confers glibenclamide sensitivity to outwardly rectifying chloride channel (ORCC) in Hi-5 insect cells.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) dependent cytoskeletal tension during lung organogenesis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) expression in human platelets: impact on mediators and mechanisms of the inflammatory response.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) functionality is dependent on coatomer protein I (COPI).
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) gene 5T allele may protect against prostate cancer: a case-control study in Chinese Han population.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) gene abnormalities in Indian males with congenital bilateral absence of vas deferens & renal anomalies.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) gene defects in patients with primary sclerosing cholangitis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation associated with a congenital bilateral absence of vas deferens.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations and risk for pancreatic adenocarcinoma.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in allergic bronchopulmonary aspergillosis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Asians with chronic pulmonary disease: a pilot study.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene mutations in North Egyptian population: implications for the genetic diagnosis in Egypt.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in pancreatitis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) gene transcripts.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Human Lung Microvascular Endothelial Cells Controls Oxidative Stress, Reactive Oxygen-Mediated Cell Signaling and Inflammatory Responses.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human bone.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) nucleotide-binding domain 1 (NBD-1) and CFTR truncated within NBD-1 target to the epithelial plasma membrane and increase anion permeability.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators protect G551D but not ?F508 CFTR from thermal instability.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) prevents apoptosis induced by hydrogen peroxide in basilar artery smooth muscle cells.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) regulates embryonic organizer formation during zebrafish early embryogenesis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR) regulates the production of osteoprotegerin (OPG) and prostaglandin (PG) E2 in human bone.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Ubiquitylation as a Novel Pharmaceutical Target for Cystic Fibrosis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR)-mediated residual chloride secretion does not protect against early chronic Pseudomonas aeruginosa infection in F508del homozygous cystic fibrosis patients.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR): CLOSED AND OPEN STATE CHANNEL MODELS.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator (CFTR): Making an ion channel out of an active transporter structure.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator activates water conductance in Xenopus oocytes.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator activation by cAMP-independent mechanisms.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator activation by roflumilast contributes to therapeutic benefit in chronic bronchitis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator activation by the solvent ethanol: implications for topical drug delivery.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator activation is reduced in the small intestine of Na+/H+ exchanger 3 regulatory factor 1 (NHERF-1)- but Not NHERF-2-deficient mice.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator activation stimulates endosome fusion in vivo.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator ameliorates lipopolysaccharide-induced acute lung injury by inhibiting autophagy through PI3K/AKT/mTOR pathway in mice.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator and adenosine triphosphate.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator and caveolin-1 regulate epithelial cell internalization of Pseudomonas aeruginosa.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator and H+ permeability in regulation of Golgi pH.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator and Na+ channel subunits mRNA transcripts, and Cl- efflux, show a different distribution in rat duodenum and colon.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator and obstructive azoospermia.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator and SLC26 transporters in HCO3(-) secretion by pancreatic duct cells.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator and the etiology and pathogenesis of cystic fibrosis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator and the outwardly rectifying chloride channel: a relationship between two chloride channels expressed in epithelial cells.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator as a model substrate to study endoplasmic reticulum protein quality control in mammalian cells.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator Attaches Tumor Suppressor PTEN to the Membrane and Promotes Anti Pseudomonas aeruginosa Immunity.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator biomarkers in 'real life': can we evaluate individual efficacy of cystic fibrosis transmembrane conductance regulator therapy?
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator Channel Dysfunction in Non-cystic Fibrosis Bronchiectasis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator chloride channel blockers: Pharmacological, biophysical and physiological relevance.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator Cl- channels with R domain deletions and translocations show phosphorylation-dependent and -independent activity.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator contributes to reacidification of alkalinized lysosomes in RPE cells.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator controls lung proteasomal degradation and nuclear factor-kappaB activity in conditions of oxidative stress.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator currents in guinea pig pancreatic duct cells: inhibition by bicarbonate ions.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator deficiency exacerbates islet cell dysfunction after beta-cell injury.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator degradation depends on the lectins Htm1p/EDEM and the Cdc48 protein complex in yeast.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator degradation: cross-talk between the ubiquitylation and SUMOylation pathways.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator differentially regulates human and mouse epithelial sodium channels in Xenopus oocytes.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator does not affect neutrophil migration across cystic fibrosis airway epithelial monolayers.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator dysfunction and its treatment.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator dysfunction in platelets drives lung hyperinflammation.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator dysfunction in VIP knockout mice.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator expression in human hypothalamus.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator expression in human spinal and sympathetic ganglia.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator facilitates ATP release by stimulating a separate ATP release channel for autocrine control of cell volume regulation.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator Function in Airway Smooth Muscle. A Novel Role in Cystic Fibrosis Airway Obstruction.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator function, not TAS2R38 gene haplotypes, predict sinus surgery in children and young adults with cystic fibrosis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator functional evaluations in a G542X+/- IVS8Tn:T7/9 patient with acute recurrent pancreatitis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator gating requires cytosolic electrolytes.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator gene abnormalities in patients with asthma and recurrent neutrophilic bronchitis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator gene mutation and lung cancer risk.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator gene mutations and glutathione S-transferase null genotypes in cystic fibrosis patients in Brazil.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator gene mutations in infertile males with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator gene mutations in severe nasal polyposis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator gene mutations: do they play a role in the aetiology of allergic bronchopulmonary aspergillosis?
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator gene polymorphisms in patients with primary sclerosing cholangitis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator gene screening and clinical correlation in Taiwanese males with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator gene variants are associated with autoimmune pancreatitis and slow response to steroid treatment.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator Genotype, Not Circulating Catecholamines, Influences Cardiovascular Function in Patients with Cystic Fibrosis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator has an altered structure when its maturation is inhibited.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator in follicular fluid and cumulus cells and its relationship with age.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator in human and mouse red blood cell membranes and its interaction with ecto-apyrase.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator in Sarcoplasmic Reticulum of Airway Smooth Muscle. Implications for Airway Contractility.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator in the endolymphatic sac of the rat.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator in the gills of the climbing perch, Anabas testudineus, is involved in both hypoosmotic regulation during seawater acclimation and active ammonia excretion during ammonia exposure.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator in the kidney: clues to its role?
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator inhibits epithelial Na+ channels carrying Liddle's syndrome mutations.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator inverts protein kinase A-mediated regulation of epithelial sodium channel single channel kinetics.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator ion channel function testing in recurrent acute pancreatitis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator is an epithelial cell receptor for clearance of Pseudomonas aeruginosa from the lung.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator is correlated closely with sperm progressive motility and normal morphology in healthy and fertile men with normal sperm parameters.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator Is Expressed in Mucin Granules from Calu-3 and Primary Human Airway Epithelial Cells.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator is found within brain ventricular epithelium and choroid plexus.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator is helpful in the distinction of extra-mammary Paget's disease from squamous cell carcinoma in situ (Bowen's disease).
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator is involved in airway epithelial wound repair.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator is involved in polyphenol-induced swelling of the endothelial glycocalyx.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator is required for protein kinase A activation of an outwardly rectified anion channel purified from bovine tracheal epithelia.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator is vital to sperm fertilizing capacity and male fertility.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator knockout mice exhibit aberrant gastrointestinal microbiota.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator mediates sulphonylurea block of the inwardly rectifying K+ channel Kir6.1.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator mediates tenogenic differentiation of tendon-derived stem cells and tendon repair: accelerating tendon injury healing by intervening in its downstream signaling.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator mediates the cyclic adenosine monophosphate-induced fluid secretion but not the inhibition of resorption in mouse gallbladder epithelium.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator modulates acute lung injury: evidence from a genetic association study*.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator modulates enteric cholinergic activities and is abnormally expressed in the enteric ganglia of patients with slow transit constipation.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator modulates neurosecretory function in pulmonary neuroendocrine cell-related tumor cell line models.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator modulates synaptic chloride homeostasis in motoneurons of the rat spinal cord during neonatal development.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator modulation by the tobacco smoke toxin acrolein.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy: A Review for the Otolaryngologist.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator modulators for cystic fibrosis: a new dawn?
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator modulators for personalized drug treatment of cystic fibrosis: progress to date.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator modulators reduce the risk of recurrent acute pancreatitis among adult patients with pancreas sufficient cystic fibrosis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator modulators: precision medicine in cystic fibrosis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator modulators: Present and future in cystic fibrosis treatment. A review.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator modulators: the end of the beginning.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator mutant F508del membrane expression rescues epithelial HCO3- secretion in murine intestine.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator mutation spectrum in patients with cystic fibrosis in Tunisia.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator mutations and polymorphisms associated with congenital bilateral absence of vas deferens in a restricted group of patients from North Africa.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator mutations at a referral center for cystic fibrosis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator mutations in azoospermic and oligospermic men and their partners.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator mutations that disrupt nucleotide binding.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator Potentiation as a Therapeutic Strategy for Pulmonary Edema: A Proof-of-Concept Study in Pigs.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator protein (CFTR) expression in the developing human brain: comparative immunohistochemical study between patients with normal and mutated CFTR.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator protein expression in brain.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator protein expression in the male excretory duct system during development.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator protein expression rate in healthy spermatozoa is not correlated with ovum fertilisation rate.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator protein: what is its role in cystic fibrosis?
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator Recruitment to Phagosomes in Neutrophils.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator Reduces Microtubule-Dependent Campylobacter jejuni Invasion.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator regulates epithelial cell response to Aspergillus and resultant pulmonary inflammation.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator regulates luminal Cl-/HCO3- exchange in mouse submandibular and pancreatic ducts.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator splice variants are not conserved and fail to produce chloride channels.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator trafficking is mediated by the COPI coat in epithelial cells.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator trafficking modulates the barrier function of airway epithelial cell monolayers.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator with a shortened R domain rescues the intestinal phenotype of CFTR-/- mice.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator-associated ATP release is controlled by a chloride sensor.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator-dependent bicarbonate entry controls rat cardiomyocyte ATP release via pannexin1 through mitochondrial signalling and caspase activation.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator-dependent up-regulation of Kir1.1 (ROMK) renal K+ channels by the epithelial sodium channel.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator-emerging regulator of cancer.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator-independent Phagosomal Acidification in Macrophages.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator-mediated corneal epithelial cell ingestion of Pseudomonas aeruginosa is a key component in the pathogenesis of experimental murine keratitis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator-modifying medications: the future of cystic fibrosis treatment.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator-mRNA delivery: a novel alternative for cystic fibrosis gene therapy.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator. Implications in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator. Permeant ions find the pore.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator. Physical basis for lyotropic anion selectivity patterns.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator. Structure and function of an epithelial chloride channel.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator: a chloride channel gated by ATP binding and hydrolysis.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator: a chloride channel with novel regulation.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator: A Molecular Model Defines the Architecture of the Anion Conduction Path and Locates a "Bottleneck" in the Pore.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator: A Possible New Target for Photodynamic Therapy Enhances Wound Healing.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator: Actin(g) as a master regulator of cholangiocyte function.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator: expression and helicity of a double membrane-spanning segment.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator: nucleotide binding to a synthetic peptide.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator: solution structures of peptides based on the Phe508 region, the most common site of disease-causing DeltaF508 mutation.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator: temperature-dependent cysteine reactivity suggests different stable conformers of the conduction pathway.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator: the first nucleotide binding fold targets the membrane with retention of its ATP binding function.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator: the NBF1+R (nucleotide-binding fold 1 and regulatory domain) segment acting alone catalyses a Co2+/Mn2+/Mg2+-ATPase activity markedly inhibited by both Cd2+ and the transition-state analogue orthovanadate.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator: the purified NBF1+R protein interacts with the purified NBF2 domain to form a stable NBF1+R/NBF2 complex while inducing a conformational change transmitted to the C-terminal region.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator: Using Differential Reactivity toward Channel-Permeant and Channel-Impermeant Thiol-Reactive Probes To Test a Molecular Model for the Pore.
Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulators (CFTR) in biliary epithelium of patients with hepatolithiasis.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator (CFTR) DeltaF508 mutation and 5T allele in patients with chronic pancreatitis and exocrine pancreatic cancer. PANKRAS II Study Group.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator (CFTR) in human trophoblast BeWo cells and its relation to cell migration.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator fragments with the Phe508 deletion exert a dual allosteric control over the master kinase CK2.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator gene (CFTR) is associated with abnormal enamel formation.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator gene carrier status is a risk factor for young onset pancreatic adenocarcinoma.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator haplotypes in households of patients with cystic fibrosis.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator inhibitors CFTR(inh)-172 and GlyH-101 target mitochondrial functions, independently of chloride channel inhibition.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator missing the first four transmembrane segments increases wild type and DeltaF508 processing.
Cystic Fibrosis
Cystic Fibrosis Transmembrane Regulator Modulators: Implications for the Management of Depression and Anxiety in Cystic Fibrosis.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator mRNA expression relative to ion-nutrient transport in spontaneously differentiating human intestinal CaCo-2 epithelial cells.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator mutations and pancreatic disease: closing the gap between genotype and phenotype.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator potentiators as promising cystic fibrosis therapies.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator protein mutations: 'class' opportunity for novel drug innovation.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator regulates uptake of sphingoid base phosphates and lysophosphatidic acid: modulation of cellular activity of sphingosine 1-phosphate.
Cystic Fibrosis
Cystic fibrosis with non-G551D gating mutations in Italy: Epidemiology and clinical characteristics.
Cystic Fibrosis
Cystic fibrosis with three mutations in the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Cystic fibrosis year in review 2019: Section 3 multisystem-based care and research.
Cystic Fibrosis
Cystic Fibrosis, Cystic Fibrosis Transmembrane Conductance Regulator and Drugs: Insights from Cellular Trafficking.
Cystic Fibrosis
Cystic fibrosis-associated mutations at arginine 347 alter the pore architecture of CFTR. Evidence for disruption of a salt bridge.
Cystic Fibrosis
Cystic Fibrosis-Related Diabetes: Pathophysiology and Therapeutic Challenges.
Cystic Fibrosis
Cystic Fibrosis-Related Pancreatic Cysts Decrease in Size and Number Upon Treatment With Cystic Fibrosis Transmembrane Conductance Regulator Modulators.
Cystic Fibrosis
Cystic fibrosis-type mutational analysis in the ATP-binding cassette transporter signature of human P-glycoprotein MDR1.
Cystic Fibrosis
Cystic fibrosis. 4. Abnormalities of airway epithelial function and the implications of the discovery of the cystic fibrosis gene.
Cystic Fibrosis
Cystic fibrosis: a brief look at some highlights of a decade of research focused on elucidating and correcting the molecular basis of the disease.
Cystic Fibrosis
Cystic fibrosis: a new target for 4-Imidazo[2,1-b]thiazole-1,4-dihydropyridines.
Cystic Fibrosis
Cystic Fibrosis: A Novel Pharmacologic Approach to Cystic Fibrosis Transmembrane Regulator Modulation Therapy.
Cystic Fibrosis
Cystic Fibrosis: A Simple and Customized Strategy for Genetic Screening Able to Detect Over 90% of Identified Mutated Alleles in Brazilian Newborns.
Cystic Fibrosis
Cystic Fibrosis: Emergence of Highly Effective Targeted Therapeutics and Potential Clinical Implications.
Cystic Fibrosis
Cystic fibrosis: exploiting its genetic basis in the hunt for new therapies.
Cystic Fibrosis
Cystic fibrosis: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases.
Cystic Fibrosis
Cystic Fibrosis: Overview of the Current Development Trends and Innovative Therapeutic Strategies.
Cystic Fibrosis
Cystic fibrosis: Physiopathology and the latest pharmacological treatments.
Cystic Fibrosis
Cystic fibrosis: S158N (605G --> A) is a rare genetic variant found in coupling with deltaF508.
Cystic Fibrosis
Cystic fibrosis: the impact of analytical technology for genotype-phenotype studies.
Cystic Fibrosis
Cystic Fibrosis: Translating Molecular Mechanisms into Effective Therapies.
Cystic Fibrosis
Cystic-fibrosis-like disease unrelated to the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Cytochrome P450 3A4 induction: lumacaftor versus ivacaftor potentially resulting in significantly reduced plasma concentration of ivacaftor.
Cystic Fibrosis
Cytogenetic analysis of azoospermic patients: karyotype comparison of peripheral blood lymphocytes and testicular tissue.
Cystic Fibrosis
Cytokine dysregulation in activated cystic fibrosis (CF) peripheral lymphocytes.
Cystic Fibrosis
Cytokine pattern in cystic fibrosis patients during antibiotic therapy and gene therapy using adenoviral vector.
Cystic Fibrosis
Cytokine-Regulation of Na(+)-K(+)-Cl(-) Cotransporter 1 and Cystic Fibrosis Transmembrane Conductance Regulator-Potential Role in Pulmonary Inflammation and Edema Formation.
Cystic Fibrosis
Cytokine-stimulated nitric oxide production inhibits adenylyl cyclase and cAMP-dependent secretion in cholangiocytes.
Cystic Fibrosis
Cytological characterization of apatitic calcium phosphate structures in bronchial epithelial tissue cultured from a child with cystic fibrosis (deltaF508).
Cystic Fibrosis
Cytoplasmic loop three of cystic fibrosis transmembrane conductance regulator contributes to regulation of chloride channel activity.
Cystic Fibrosis
Cytoplasmic pathway followed by chloride ions to enter the CFTR channel pore.
Cystic Fibrosis
Cytoprotective antioxidant function of tyrosine and tryptophan residues in transmembrane proteins.
Cystic Fibrosis
Cytoskeleton regulators CAPZA2 and INF2 associate with CFTR to control its plasma membrane levels under EPAC1 activation.
Cystic Fibrosis
Cytosolic pH regulates GCl through control of phosphorylation states of CFTR.
Cystic Fibrosis
Dab2 is a key regulator of endocytosis and post-endocytic trafficking of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor.
Cystic Fibrosis
Dataset of differential lipid raft and global proteomes of SILAC-labeled cystic fibrosis cells upon TNF -? stimulation.
Cystic Fibrosis
DCPIB is a novel selective blocker of I(Cl,swell) and prevents swelling-induced shortening of guinea-pig atrial action potential duration.
Cystic Fibrosis
Deactivation of CFTR-Cl conductance by endogenous phosphatases in the native sweat duct.
Cystic Fibrosis
Decreased expression of cystic fibrosis transmembrane conductance regulator impairs sperm quality in aged men.
Cystic Fibrosis
Decreased expression of peroxisome proliferator activated receptor gamma in cftr-/- mice.
Cystic Fibrosis
Decreased expression of the CFTR protein in remodeled human nasal epithelium from non-cystic fibrosis patients.
Cystic Fibrosis
Decreased expression of the cystic fibrosis transmembrane conductance regulator protein in remodeled airway epithelium from lung transplanted patients.
Cystic Fibrosis
Decreased Peroxisome Proliferator Activated Receptor alpha Is Associated with Bile Duct Injury in Cystic Fibrosis Transmembrane Conductance Regulator-/- Mice.
Cystic Fibrosis
Decreased soluble adenylyl cyclase activity in cystic fibrosis is related to defective apical bicarbonate exchange and affects ciliary beat frequency regulation.
Cystic Fibrosis
Deep interactome profiling of membrane proteins by co-interacting protein identification technology.
Cystic Fibrosis
Defective CFTR apical endocytosis and enterocyte brush border in myosin VI-deficient mice.
Cystic Fibrosis
Defective CFTR Expression and Function Are Detectable in Blood Monocytes: Development of a New Blood Test for Cystic Fibrosis.
Cystic Fibrosis
Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition.
Cystic Fibrosis
Defective CFTR promotes intestinal proliferation via inhibition of the hedgehog pathway during cystic fibrosis.
Cystic Fibrosis
Defective CFTR-dependent CREB activation results in impaired spermatogenesis and azoospermia.
Cystic Fibrosis
Defective cholinergic Cl(-) secretion and detection of K(+) secretion in rectal biopsies from cystic fibrosis patients.
Cystic Fibrosis
Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis.
Cystic Fibrosis
Defective exocytosis and processing of insulin in a cystic fibrosis mouse model.
Cystic Fibrosis
Defective formation of PKA/CnA-dependent annexin 2-S100A10/CFTR complex in DeltaF508 cystic fibrosis cells.
Cystic Fibrosis
Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein.
Cystic Fibrosis
Defective Innate Immunity and Hyper-Inflammation in Newborn CFTR-Knockout Ferret Lungs.
Cystic Fibrosis
DEFECTIVE ORGANELLAR ACIDIFICATION AS A CAUSE OF CYSTIC FIBROSIS LUNG DISEASE - RE-EXAMINATION OF A RECURRING HYPOTHESIS.
Cystic Fibrosis
Defective regulation of outwardly rectifying Cl- channels by protein kinase A corrected by insertion of CFTR.
Cystic Fibrosis
Defective regulatory volume decrease in human cystic fibrosis tracheal cells because of altered regulation of intermediate conductance Ca2+-dependent potassium channels.
Cystic Fibrosis
Defective trafficking and function of KATP channels caused by a sulfonylurea receptor 1 mutation associated with persistent hyperinsulinemic hypoglycemia of infancy.
Cystic Fibrosis
Defects in Gallbladder Emptying and Bile Acid Homeostasis in Mice with Cystic Fibrosis Transmembrane Conductance Regulator Deficiencies.
Cystic Fibrosis
Defects in processing and trafficking of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Defects in processing and trafficking of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Defining a mutational panel and predicting the prevalence of cystic fibrosis in oman.
Cystic Fibrosis
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Defining the role of CFTR channel blocker and ClC-2 activator in DNBS induced gastrointestinal inflammation.
Cystic Fibrosis
Definition of a "functional R domain" of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Deformation-induced ATP release from red blood cells requires CFTR activity.
Cystic Fibrosis
Dehydrocostuslactone, a sesquiterpene lactone activates wild-type and ?F508 mutant CFTR chloride channel.
Cystic Fibrosis
Delayed association of the NADPH oxidase complex with macrophage vacuoles containing the opportunistic pathogen Burkholderia cenocepacia.
Cystic Fibrosis
Deleterious impact of Pseudomonas aeruginosa on cystic fibrosis transmembrane conductance regulator function and rescue in airway epithelial cells.
Cystic Fibrosis
Deletion and insertion mutations in short tandem repeats in the coding regions of human genes.
Cystic Fibrosis
Deletion of cftr Leads to an Excessive Neutrophilic Response and Defective Tissue Repair in a Zebrafish Model of Sterile Inflammation.
Cystic Fibrosis
Deletion of exons 16-17b of CFTR is frequently identified in Korean patients with cystic fibrosis.
Cystic Fibrosis
Deletion of Phe508 in the first nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator increases its affinity for the heat shock cognate 70 chaperone.
Cystic Fibrosis
Deletion of phenylalanine 508 causes attenuated phosphorylation-dependent activation of CFTR chloride channels.
Cystic Fibrosis
Deletion of Phenylalanine 508 in the First Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator Increases Conformational Exchange and Inhibits Dimerization.
Cystic Fibrosis
Deletion of the ubiquitin ligase Nedd4L in lung epithelia causes cystic fibrosis-like disease.
Cystic Fibrosis
Delivery of purified, functional CFTR to epithelial cells in vitro using influenza hemagglutinin.
Cystic Fibrosis
Delta F508 CFTR localizes in the endoplasmic reticulum-Golgi intermediate compartment in cystic fibrosis cells.
Cystic Fibrosis
Delta F508 CFTR pool in the endoplasmic reticulum is increased by calnexin overexpression.
Cystic Fibrosis
Delta F508 mutation of cystic fibrosis gene is not found in chronic bronchitis with severe obstruction in Japan.
Cystic Fibrosis
DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers.
Cystic Fibrosis
DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis.
Cystic Fibrosis
Demographics of the UK cystic fibrosis population: implications for neonatal screening.
Cystic Fibrosis
Demonstration of phosphoryl-group transfer indicates that the ABC transporter CFTR exhibits adenylate kinase activity.
Cystic Fibrosis
Demonstration that CFTR is a chloride channel by alteration of its anion selectivity.
Cystic Fibrosis
Denaturing high performance liquid chromatography (DHPLC) used in the detection of germline and somatic mutations.
Cystic Fibrosis
Denopamine stimulates alveolar fluid clearance via cystic fibrosis transmembrane conductance regulator in rat lungs.
Cystic Fibrosis
Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function.
Cystic Fibrosis
Dependence of cAMP meditated increases in Cl(-) and HCO(3)(-) permeability on CFTR in bovine corneal endothelial cells.
Cystic Fibrosis
Depletion of the ubiquitin-binding adaptor molecule SQSTM1/p62 from macrophages harboring cftr ?F508 mutation improves the delivery of Burkholderia cenocepacia to the autophagic machinery.
Cystic Fibrosis
Deregulated expression and function of CFTR and Cl- secretion after activation of the Ras and Src/PyMT pathways in Caco-2 cells.
Cystic Fibrosis
Derivation of adult canine intestinal organoids for translational research in gastroenterology.
Cystic Fibrosis
Derivation of lung epithelium from human cord blood-derived mesenchymal stem cells.
Cystic Fibrosis
Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutants.
Cystic Fibrosis
Description of a symptomless cystic fibrosis L346P/M348K compound heterozygous Cypriot individual.
Cystic Fibrosis
Design and characterization of a membrane protein unfolding platform in lipid bilayers.
Cystic Fibrosis
Designing Clinical Trials for Anti-Inflammatory Therapies in Cystic Fibrosis.
Cystic Fibrosis
Destabilization of the transmembrane domain induces misfolding in a phenotypic mutant of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Detailed analysis of structures and formulations of cationic lipids for efficient gene transfer to the lung.
Cystic Fibrosis
DETANO and nitrated lipids increase chloride secretion across lung airway cells.
Cystic Fibrosis
Detecting Common CFTR Mutations by Reverse Dot Blot Hybridization Method in Cystic Fibrosis First Report from Northern Iran.
Cystic Fibrosis
Detection of 98.5% of the mutations in 200 Belgian cystic fibrosis alleles by reverse dot-blot and sequencing of the complete coding region and exon/intron junctions of the CFTR gene.
Cystic Fibrosis
Detection of a de novo R1066H mutation in an Italian patient affected by cystic fibrosis.
Cystic Fibrosis
Detection of adenylate cyclase-coupled receptors in Xenopus oocytes by coexpression with cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Detection of an Apparent Homozygous 3120G>A Cystic Fibrosis Mutation on a Routine Carrier Screen.
Cystic Fibrosis
Detection of CFTR gene mutations in patients suffering from chronic bronchitis.
Cystic Fibrosis
Detection of CFTR mutations using temporal temperature gradient gel electrophoresis.
Cystic Fibrosis
Detection of cystic fibrosis mutations by peptide mass signature genotyping.
Cystic Fibrosis
Detection of Cystic Fibrosis Serological Biomarkers Using a T7 Phage Display Library.
Cystic Fibrosis
Detection of cystic fibrosis transmembrane conductance regulator (CFTR) gene rearrangements enriches the mutation spectrum in congenital bilateral absence of the vas deferens and impacts on genetic counselling.
Cystic Fibrosis
Detection of cystic fibrosis transmembrane conductance regulator ?F508 gene mutation using a paper-based nucleic acid hybridization assay and a smartphone camera.
Cystic Fibrosis
Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials.
Cystic Fibrosis
Detection of delta F508 mutation of the cystic fibrosis gene by matrix-assisted laser desorption/ionization mass spectrometry.
Cystic Fibrosis
Detection of F508del mutation in cystic fibrosis transmembrane conductance regulator gene mutation among Malays.
Cystic Fibrosis
Detection of five novel mutations of the cystic fibrosis transmembrane regulator (CFTR) gene in Pakistani patients with cystic fibrosis: Y569D, Q98X, 296+12(T>C), 1161delC and 621+2(T>C).
Cystic Fibrosis
Detection of five rare cystic fibrosis mutations peculiar to Southern Italy: implications in screening for the disease and phenotype characterization for patients with homozygote mutations.
Cystic Fibrosis
Detection of large rearrangements in the cystic fibrosis transmembrane conductance regulator gene by multiplex ligation-dependent probe amplification assay when sequencing fails to detect two disease-causing mutations.
Cystic Fibrosis
Detection of modifier loci influencing the lung phenotype of cystic fibrosis knockout mice.
Cystic Fibrosis
Detection of more than 50 different CFTR mutations in a large group of German cystic fibrosis patients.
Cystic Fibrosis
Detection of point mutations by capillary electrophoresis in liquid polymers in temporal thermal gradients.
Cystic Fibrosis
Detection of the deltaF508 (F508del) mutation of the cystic fibrosis gene by surface plasmon resonance and biosensor technology.
Cystic Fibrosis
Detection of the DeltaF508 mutation in the CFTR gene by means of time-resolved fluorescence methods.
Cystic Fibrosis
Detergent binding explains anomalous SDS-PAGE migration of membrane proteins.
Cystic Fibrosis
Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods.
Cystic Fibrosis
Determination of the functional unit of the cystic fibrosis transmembrane conductance regulator chloride channel. One polypeptide forms one pore.
Cystic Fibrosis
Determination of the relative contribution of three genes-the cystic fibrosis transmembrane conductance regulator gene, the cationic trypsinogen gene, and the pancreatic secretory trypsin inhibitor gene-to the etiology of idiopathic chronic pancreatitis.
Cystic Fibrosis
Determining the pathogenicity of CFTR missense variants: Multiple comparisons of in silico predictors and variant annotation databases.
Cystic Fibrosis
Development and analysis of recombinant adenoviruses for gene therapy of cystic fibrosis.
Cystic Fibrosis
Development and characterization of secretin-stimulated secretion of cultured rat cholangiocytes.
Cystic Fibrosis
Development and characterization of synthetic antibodies binding to the cystic fibrosis conductance regulator.
Cystic Fibrosis
Development and evaluation of a PCR-based, line probe assay for the detection of 58 alleles in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
Development and functional characterization of extrahepatic cholangiocyte lines from normal rats.
Cystic Fibrosis
Development and regulation of chloride homeostasis in the central nervous system.
Cystic Fibrosis
Development and validation of a screening test for 12 common mutations of the cystic fibrosis CFTR gene.
Cystic Fibrosis
Development and Validation of HTS Flux Assay for Endogenously Expressed Chloride Channels in a CHO-K1 Cell Line.
Cystic Fibrosis
DEVELOPMENT OF A POLARIZED PANCREATIC DUCTULAR CELL EPITHELIA FOR PHYSIOLOGICAL STUDIES.
Cystic Fibrosis
Development of allele-specific multiplex PCR to determine the length of poly-T in intron 8 of CFTR.
Cystic Fibrosis
Development of an epithelium-specific expression cassette with human DNA regulatory elements for transgene expression in lung airways.
Cystic Fibrosis
Development of Automated Patch Clamp Technique to Investigate CFTR Chloride Channel Function.
Cystic Fibrosis
Development of elexacaftor - tezacaftor - ivacaftor: Highly effective CFTR modulation for the majority of people with Cystic Fibrosis.
Cystic Fibrosis
Development of intestinal ion-transporting mechanisms during smoltification and seawater acclimation in Atlantic salmon Salmo salar.
Cystic Fibrosis
Development of PEGylated PLGA nanoparticle for controlled and sustained drug delivery in cystic fibrosis.
Cystic Fibrosis
Development of structural marker peptides for cystic fibrosis transmembrane conductance regulator in cell plasma membrane by reversed-footprinting mass spectrometry.
Cystic Fibrosis
Development of substituted Benzo[c]quinolizinium compounds as novel activators of the cystic fibrosis chloride channel.
Cystic Fibrosis
Development, multiplexing, and application of ARMS tests for common mutations in the CFTR gene.
Cystic Fibrosis
Developmental differences of cystic fibrosis transmembrane conductance regulator functional expression in isolated rat fetal distal airway epithelial cells.
Cystic Fibrosis
Developmental expression of a mucinlike glycoprotein (MUCLIN) in pancreas and small intestine of CF mice.
Cystic Fibrosis
Developmental expression patterns of CFTR in ferret tracheal surface airway and submucosal gland epithelia.
Cystic Fibrosis
Diabetic cornea wounds produce significantly weaker electric signals that may contribute to impaired healing.
Cystic Fibrosis
Diagnosis of cystic fibrosis in adulthood and eligibility for novel CFTR modulator therapy.
Cystic Fibrosis
Diagnosis of cystic fibrosis in the kindred of an infant with CFTR-related metabolic syndrome: importance of follow-up that includes monitoring sweat chloride concentrations over time.
Cystic Fibrosis
Diagnosis, follow-up and treatment of cystic fibrosis-related liver disease.
Cystic Fibrosis
Diagnostic contribution of molecular analysis of the cystic fibrosis transmembrane conductance regulator gene in patients suspected of having mild or atypical cystic fibrosis.
Cystic Fibrosis
Diagnostic tools and CFTR functional assays in cystic fibrosis: utility and availability in Switzerland.
Cystic Fibrosis
Diagnostic usefulness of the polymorphism of the GT dinucleotide and the polythymidine tract in intron 8 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
Dibasic protein kinase A sites regulate bursting rate and nucleotide sensitivity of the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Diet-Induced Dysbiosis and Genetic Background Synergize With Cystic Fibrosis Transmembrane Conductance Regulator Deficiency to Promote Cholangiopathy in Mice.
Cystic Fibrosis
Dietary genistein stimulates anion secretion across female murine intestine.
Cystic Fibrosis
Diethylstilbestrol, a Novel ANO1 Inhibitor, Exerts an Anticancer Effect on Non-Small Cell Lung Cancer via Inhibition of ANO1.
Cystic Fibrosis
Diets high in heat-treated soybean meal reduce the histamine-induced epithelial response in the colon of weaned piglets and increase epithelial catabolism of histamine.
Cystic Fibrosis
Differences between cystic fibrosis transmembrane conductance regulator and HisP in the interaction with the adenine ring of ATP.
Cystic Fibrosis
Differences in gene mutations between Chinese and Caucasian cystic fibrosis patients.
Cystic Fibrosis
Differences in the protein-kinase-A-dependent regulation of CFTR Cl- channels and Na+-K+ pumps in guinea-pig ventricular myocytes.
Cystic Fibrosis
Different activation mechanisms of cystic fibrosis transmembrane conductance regulator expressed in Xenopus laevis oocytes.
Cystic Fibrosis
Different CFTR mutational spectrum in alcoholic and idiopathic chronic pancreatitis?
Cystic Fibrosis
Different cystic fibrosis transmembrane conductance regulator mutations in chinese men with congenital bilateral absence of vas deferens and other acquired obstructive azoospermia.
Cystic Fibrosis
Different functional roles for K+ channel subtypes in regulating small intestinal glucose and ion transport.
Cystic Fibrosis
Different immunohistochemical localization for TMEM16A and CFTR in acinar and ductal cells of rat major salivary glands and exocrine pancreas.
Cystic Fibrosis
Different p97/VCP complexes function in retrotranslocation step of mammalian Er-associated degradation (ERAD).
Cystic Fibrosis
Different SUMO paralogues determine the fate of wild-type and mutant CFTRs: biogenesis versus degradation.
Cystic Fibrosis
Differential acidic pH sensitivity of delta F508 CFTR Cl- channel activity in lipid bilayers.
Cystic Fibrosis
Differential activation of the HCO(3) conductance through the cystic fibrosis transmembrane conductance regulator anion channel by genistein and forskolin in murine duodenum.
Cystic Fibrosis
Differential cellular expression of cystic fibrosis transmembrane regulator in human reproductive tissues. Clues for the infertility in patients with cystic fibrosis.
Cystic Fibrosis
Differential contribution of cis-regulatory elements to higher order chromatin structure and expression of the CFTR locus.
Cystic Fibrosis
Differential coupling of G protein alpha subunits to seven-helix receptors expressed in Xenopus oocytes.
Cystic Fibrosis
Differential effects of aldosterone and vasopressin on chloride fluxes in transimmortalized mouse cortical collecting duct cells.
Cystic Fibrosis
Differential effects of Matrigel and its components on functional activity of CFTR and ENaC in mouse endometrial epithelial cells.
Cystic Fibrosis
Differential effects of polycationic protein on Cl- secretory and Na+ absorptive airways.
Cystic Fibrosis
Differential effects on the miRNome of the treatment of human airway epithelial Calu-3 cells with peptide-nucleic acids (PNAs) targeting microRNAs miR-101-3p and miR-145-5p: Next generation sequencing datasets.
Cystic Fibrosis
Differential expression and localization of CFTR and ENaC in mouse endometrium during pre-implantation.
Cystic Fibrosis
Differential expression of gill Na+,K+-ATPase alpha- and beta-subunits, Na+,K+,2Cl- cotransporter and CFTR anion channel in juvenile anadromous and landlocked Atlantic salmon Salmo salar.
Cystic Fibrosis
Differential expression of intestinal ion transporters and water channel aquaporins in young piglets challenged with enterotoxigenic
Cystic Fibrosis
Differential function of the two nucleotide binding domains on cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Differential interactions of nucleotides at the two nucleotide binding domains of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Differential localization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis airway epithelium.
Cystic Fibrosis
Differential regulation of CFTRDeltaF508 degradation by ubiquitin ligases gp78 and Hrd1.
Cystic Fibrosis
Differential regulation of cystic fibrosis transmembrane conductance regulator and Na+,K+ -ATPase in gills of striped bass, Morone saxatilis: effect of salinity and hormones.
Cystic Fibrosis
Differential regulation of cystic fibrosis transmembrane conductance regulator by interferon gamma in mast cells and epithelial cells.
Cystic Fibrosis
Differential regulation of inflammation by inflammatory mediators in cystic fibrosis lung epithelial cells.
Cystic Fibrosis
Differential regulation of single CFTR channels by PP2C, PP2A, and other phosphatases.
Cystic Fibrosis
Differential regulations between adenosine triphosphate (ATP)- and uridine triphosphate-induced Cl(-) secretion in bovine tracheal epithelium. Direct stimulation of P1-like receptor by ATP.
Cystic Fibrosis
Differential sensitivity of plant and yeast MRP (ABCC)-mediated organic anion transport processes towards sulfonylureas.
Cystic Fibrosis
Differential sensitivity of the cystic fibrosis (CF)-associated mutants G551D and G1349D to potentiators of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel.
Cystic Fibrosis
Differential stimulation of cytosolic phospholipase A2 by bradykinin in human cystic fibrosis cell lines.
Cystic Fibrosis
Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR.
Cystic Fibrosis
Differentiation of adult rat bone marrow stem cells into epithelial progenitor cells in culture.
Cystic Fibrosis
Differentiation of human bronchial epithelial cells: role of hydrocortisone in development of ion transport pathways involved in mucociliary clearance.
Cystic Fibrosis
Differentiation of immortalized epithelial cells derived from cystic fibrosis airway submucosal glands.
Cystic Fibrosis
Diffusional mobility of the cystic fibrosis transmembrane conductance regulator mutant, delta F508-CFTR, in the endoplasmic reticulum measured by photobleaching of GFP-CFTR chimeras.
Cystic Fibrosis
Dimeric cystic fibrosis transmembrane conductance regulator exists in the plasma membrane.
Cystic Fibrosis
Diminished self-chaperoning activity of the DeltaF508 mutant of CFTR results in protein misfolding.
Cystic Fibrosis
Dinucleotide (CA/GT) repeat polymorphism in intron 17B of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-diallyl-8-cyclohexylxanthine (DAX).
Cystic Fibrosis
Direct and indirect effects of mutations at the outer mouth of the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Cystic Fibrosis
Direct Binding of the Corrector VX-809 to Human CFTR NBD1: Evidence of an Allosteric Coupling between the Binding Site and the NBD1:CL4 Interface.
Cystic Fibrosis
Direct block of the cystic fibrosis transmembrane conductance regulator Cl(-) channel by butyrate and phenylbutyrate.
Cystic Fibrosis
Direct block of the cystic fibrosis transmembrane conductance regulator Cl(-) channel by niflumic acid.
Cystic Fibrosis
Direct comparison of the functional roles played by different transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Cystic Fibrosis
Direct effects of 9-anthracene compounds on cystic fibrosis transmembrane conductance regulator gating.
Cystic Fibrosis
Direct interaction of a CFTR potentiator and a CFTR corrector with phospholipid bilayers.
Cystic Fibrosis
Direct interaction with filamins modulates the stability and plasma membrane expression of CFTR.
Cystic Fibrosis
Direct Measurement of Trafficking of the Cystic Fibrosis Transmembrane Conductance Regulator to the Cell Surface and Binding to a Chemical Chaperone.
Cystic Fibrosis
Direct sensing of intracellular pH by the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel.
Cystic Fibrosis
Direct visualization of cystic fibrosis transmembrane regulator mutations in the clinical laboratory setting.
Cystic Fibrosis
Directed differentiation of cholangiocytes from human pluripotent stem cells.
Cystic Fibrosis
Discovery and development of antisecretory drugs for treating diarrheal diseases.
Cystic Fibrosis
Discovery of 1,2,3,4-tetrahydroisoquinoline-3-carboxylic acid diamides that increase CFTR mediated chloride transport.
Cystic Fibrosis
Discovery of 4-[(2R,4R)-4-({[1-(2,2-Difluoro-1,3-benzodioxol-5-yl)cyclopropyl]carbonyl}amino)-7-(difluoromethoxy)-3,4-dihydro-2H-chromen-2-yl]benzoic Acid (ABBV/GLPG-2222), a Potent Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Corrector for the Treatment of Cystic Fibrosis.
Cystic Fibrosis
Discovery of ABBV/GLPG-3221, a Potent Corrector of CFTR for the Treatment of Cystic Fibrosis.
Cystic Fibrosis
Discovery of alpha-aminoazaheterocycle-methylglyoxal adducts as a new class of high-affinity inhibitors of cystic fibrosis transmembrane conductance regulator chloride channels.
Cystic Fibrosis
Discovery of Clinically Approved Agents That Promote Suppression of CFTR Nonsense Mutations.
Cystic Fibrosis
Discovery of GLPG2451, a Novel Once Daily Potentiator for the Treatment of Cystic Fibrosis.
Cystic Fibrosis
Discovery of glycine hydrazide pore-occluding CFTR inhibitors: mechanism, structure-activity analysis, and in vivo efficacy.
Cystic Fibrosis
Discovery of Icenticaftor (QBW251), a Cystic Fibrosis Transmembrane Conductance Regulator Potentiator with Clinical Efficacy in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Cystic Fibrosis
Discovery of Multitarget Agents Active as Broad-Spectrum Antivirals and Correctors of Cystic Fibrosis Transmembrane Conductance Regulator for Associated Pulmonary Diseases.
Cystic Fibrosis
Discovery of N-(3-Carbamoyl-5,5,7,7-tetramethyl-5,7-dihydro-4H-thieno[2,3-c]pyran-2-yl)-lH-pyrazole-5-carboxamide (GLPG1837), a Novel Potentiator Which Can Open Class III Mutant Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Channels to a High Extent.
Cystic Fibrosis
Discovery of novel VX-809 hybrid derivatives as F508del-CFTR correctors by molecular modeling, chemical synthesis and biological assays.
Cystic Fibrosis
Discovery of Pyrrolo[2,3-b]pyrazines Derivatives as Submicromolar Affinity Activators of Wild Type, G551D, and F508del Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channels.
Cystic Fibrosis
Discrimination between cystic fibrosis and CFTR-corrected epithelial cells by a membrane potential-sensitive probe.
Cystic Fibrosis
Disease causing mutations in the cystic fibrosis transmembrane conductance regulator determine the functional responses of alveolar macrophages.
Cystic Fibrosis
Disease-associated mutations in cytoplasmic loops 1 and 2 of cystic fibrosis transmembrane conductance regulator impede processing or opening of the channel.
Cystic Fibrosis
Disease-associated mutations in the extracytoplasmic loops of cystic fibrosis transmembrane conductance regulator do not impede biosynthetic processing but impair chloride channel stability.
Cystic Fibrosis
Disease-associated mutations in the fourth cytoplasmic loop of cystic fibrosis transmembrane conductance regulator compromise biosynthetic processing and chloride channel activity.
Cystic Fibrosis
Disease-relevant mutations alter amino acid co-evolution networks in the second nucleotide binding domain of CFTR.
Cystic Fibrosis
Disease-relevant proteostasis regulation of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Dispersion of Endoplasmic Reticulum-associated Compartments by 4-phenyl Butyric Acid in Yeast Cells.
Cystic Fibrosis
Disrupted local innervation results in less VIP expression in CF mice tissues.
Cystic Fibrosis
Disrupted post-transcriptional regulation of the cystic fibrosis transmembrane conductance regulator (CFTR) by a 5'UTR mutation is associated with a CFTR-related disease.
Cystic Fibrosis
Disruption of AtMRP4, a guard cell plasma membrane ABCC-type ABC transporter, leads to deregulation of stomatal opening and increased drought susceptibility.
Cystic Fibrosis
Disruption of biofilms and killing of Burkholderia cenocepacia from cystic fibrosis lung using an antioxidant-antibiotic combination therapy.
Cystic Fibrosis
Disruption of CFTR-dependent lipid rafts reduces bacterial levels and corneal disease in a murine model of Pseudomonas aeruginosa keratitis.
Cystic Fibrosis
Disruption of cytokeratin-8 interaction with F508del-CFTR corrects its functional defect.
Cystic Fibrosis
Disruption of interleukin-1? autocrine signaling rescues complex I activity and improves ROS levels in immortalized epithelial cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function.
Cystic Fibrosis
Disruption of monolayer integrity enables activation of a cystic fibrosis "bypass" channel in human airway epithelia.
Cystic Fibrosis
Disruption of the cystic fibrosis transmembrane conductance regulator gene in embryonic stem cells by gene targeting.
Cystic Fibrosis
Dissection of de novo membrane insertion activities of internal transmembrane segments of ATP-binding-cassette transporters: toward understanding topological rules for membrane assembly of polytopic membrane proteins.
Cystic Fibrosis
Dissection of the Role of VIMP in Endoplasmic Reticulum-Associated Degradation of CFTR?F508.
Cystic Fibrosis
Distinct Mg(2+)-dependent steps rate limit opening and closing of a single CFTR Cl(-) channel.
Cystic Fibrosis
Distinct roles for the Hsp40 and Hsp90 molecular chaperones during cystic fibrosis transmembrane conductance regulator degradation in yeast.
Cystic Fibrosis
Distinct spectrum of CFTR gene mutations in congenital absence of vas deferens.
Cystic Fibrosis
Distribution and regulation of ENaC subunit and CFTR mRNA expression in murine female reproductive tract.
Cystic Fibrosis
Distribution of aquaporin water channels AQP1 and AQP5 in the ductal system of the human pancreas.
Cystic Fibrosis
Distribution of CFTR mutations in Saguenay- Lac-Saint-Jean: proposal of a panel of mutations for population screening.
Cystic Fibrosis
Distribution of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mutations in a Cohort of Patients Residing in Palestine.
Cystic Fibrosis
Distribution patterns of the delta F508 mutation in the CFTR gene of CF-linked marker haplotypes in the German population.
Cystic Fibrosis
Disturbances of colonic ion secretion in inflammation: role of the enteric nervous system and cAMP.
Cystic Fibrosis
Disulphonic stilbene block of cystic fibrosis transmembrane conductance regulator Cl- channels expressed in a mammalian cell line and its regulation by a critical pore residue.
Cystic Fibrosis
Divergent CFTR orthologs respond differently to the channel inhibitors CFTRinh-172, glibenclamide, and GlyH-101.
Cystic Fibrosis
Diverse genotypical features and impacts on clinical course and severity of cystic fibrosis: early childhood experience.
Cystic Fibrosis
Diversified Synthetic Pathway of 1, 4-Dihydropyridines: A Class of Pharmacologically Important Molecules.
Cystic Fibrosis
DNA immobilisation procedures for surface plasmon resonance imaging (SPRI) based microarray systems.
Cystic Fibrosis
DNA Methylation Patterns Correlate with the Expression of SCNN1A, SCNN1B, and SCNN1G (Epithelial Sodium Channel, ENaC) Genes.
Cystic Fibrosis
DNA polymorphisms in potential regulatory elements of the CFTR gene alter transcription factor binding.
Cystic Fibrosis
Does cystic fibrosis neonatal screening detect atypical CF forms? Extended genetic characterization and 4-year clinical follow-up.
Cystic Fibrosis
Does epithelial sodium channel hyperactivity contribute to cystic fibrosis lung disease?
Cystic Fibrosis
Does ivacaftor interfere with the antimicrobial activity of commonly used antibiotics against Pseudomonas aeruginosa?-Results of an in vitro study.
Cystic Fibrosis
Does stimulation of NaCl secretion in in vitro perfused rectal gland tubules of squalus acanthias increase membrane capacitance?
Cystic Fibrosis
Does the {Delta}F508-CFTR mutation induce a proinflammatory response in human airway epithelial cells?
Cystic Fibrosis
Does type 2 diabetes mellitus delay renal failure in autosomal dominant polycystic kidney disease?
Cystic Fibrosis
Domain interactions in the yeast ATP binding cassette transporter Ycf1p: intragenic suppressor analysis of mutations in the nucleotide binding domains.
Cystic Fibrosis
Domain location within the cystic fibrosis transmembrane conductance regulator protein investigated by electron microscopy and gold labelling.
Cystic Fibrosis
Domain-domain associations in cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Down regulation of small intestinal ion transport in PDZK1- (CAP70/NHERF3) deficient mice.
Cystic Fibrosis
Down-regulated CFTR during aging contributes to benign prostatic hyperplasia.
Cystic Fibrosis
Down-regulation of cystic fibrosis transmembrane conductance regulator gene expression by agents that modulate intracellular divalent cations.
Cystic Fibrosis
Down-regulation of the anti-inflammatory protein annexin A1 in cystic fibrosis knock-out mice and patients.
Cystic Fibrosis
Down-regulation of volume-sensitive Cl- channels by CFTR is mediated by the second nucleotide-binding domain.
Cystic Fibrosis
Downregulation of CFTR promotes epithelial-to-mesenchymal transition and is associated with poor prognosis of breast cancer.
Cystic Fibrosis
Downregulation of epithelial sodium channel (ENaC) by CFTR co-expressed in Xenopus oocytes is independent of Cl- conductance.
Cystic Fibrosis
Drug-induced dyspnea versus cystic fibrosis exacerbation: a diagnostic dilemma.
Cystic Fibrosis
Drug-set enrichment analysis: a novel tool to investigate drug mode of action.
Cystic Fibrosis
Dual activity of aminoarylthiazoles on the trafficking and gating defects of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel caused by cystic fibrosis mutations.
Cystic Fibrosis
Dual effects of ADP and adenylylimidodiphosphate on CFTR channel kinetics show binding to two different nucleotide binding sites.
Cystic Fibrosis
Dual regulation of cardiac Na+-K+ pumps and CFTR Cl- channels by protein kinases A and C.
Cystic Fibrosis
Dual role of CFTR in cAMP-stimulated HCO3- secretion across murine duodenum.
Cystic Fibrosis
Dual roles of the sixth transmembrane segment of the CFTR chloride channel in gating and permeation.
Cystic Fibrosis
Ductular network formation by rat biliary epithelial cells in the dynamical culture with collagen gel and dimethylsulfoxide stimulation.
Cystic Fibrosis
Duodenal brush border intestinal alkaline phosphatase activity affects bicarbonate secretion in rats.
Cystic Fibrosis
Duplicated CFTR isoforms in eels diverged in regulatory structures and osmoregulatory functions.
Cystic Fibrosis
Dynamic activation of cystic fibrosis transmembrane conductance regulator by type 3 and type 4D phosphodiesterase inhibitors.
Cystic Fibrosis
Dynamic control of cystic fibrosis transmembrane conductance regulator Cl(-)/HCO3(-) selectivity by external Cl(-).
Cystic Fibrosis
Dynamic Regulation of CFTR Bicarbonate Permeability by [Cl-]i and its Role in Pancreatic Bicarbonate Secretion.
Cystic Fibrosis
Dynamic regulation of cystic fibrosis transmembrane conductance regulator by competitive interactions of molecular adaptors.
Cystic Fibrosis
Dynamically regulated CFTR expression and its functional role in cutaneous wound healing.
Cystic Fibrosis
Dynamics intrinsic to cystic fibrosis transmembrane conductance regulator function and stability.
Cystic Fibrosis
Dynamics of bacterial colonisation in the respiratory tract of patients with cystic fibrosis.
Cystic Fibrosis
Dynamics of Gene Expression Responses for Ion Transport Proteins and Aquaporins in the Gill of a Euryhaline Pupfish during Freshwater and High-Salinity Acclimation.
Cystic Fibrosis
Dysfunction of the non-neuronal cholinergic system in the airways and blood cells of patients with cystic fibrosis.
Cystic Fibrosis
Dysfunctional CFTR alters the bactericidal activity of human macrophages against Pseudomonas aeruginosa.
Cystic Fibrosis
Dysfunctional cystic fibrosis transmembrane conductance regulator inhibits phagocytosis of apoptotic cells with proinflammatory consequences.
Cystic Fibrosis
Dysfunctional Inflammation in Cystic Fibrosis Airways: From Mechanisms to Novel Therapeutic Approaches.
Cystic Fibrosis
Dysregulated signalling pathways in innate immune cells with cystic fibrosis mutations.
Cystic Fibrosis
Dysregulation of proteoglycan production by intrahepatic biliary epithelial cells bearing defective (delta-f508) cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
E3KARP mediates the association of ezrin and protein kinase A with the cystic fibrosis transmembrane conductance regulator in airway cells.
Cystic Fibrosis
Early activation of sperm by HCO(3)(-) is regulated hormonally in the murine uterus.
Cystic Fibrosis
Early acute pancreatitis in a child with compound heterozygosis ?F508/R1438W/Y1032C cystic fibrosis: a case report.
Cystic Fibrosis
Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice.
Cystic Fibrosis
Early Diagnosis and Intervention in Cystic Fibrosis: Imagining the Unimaginable.
Cystic Fibrosis
Early embryonic renal tubules of wild-type and polycystic kidney disease kidneys respond to cAMP stimulation with cystic fibrosis transmembrane conductance regulator/Na(+),K(+),2Cl(-) Co-transporter-dependent cystic dilation.
Cystic Fibrosis
Economic implications of newborn screening for cystic fibrosis: a cost of illness retrospective cohort study.
Cystic Fibrosis
EDNRA variants associate with smooth muscle mRNA levels, cell proliferation rates, and cystic fibrosis pulmonary disease severity.
Cystic Fibrosis
Effect of acupuncture on chronic bronchitis: A protocol for systematic review and meta-analysis.
Cystic Fibrosis
Effect of Alpha-1 Antitrypsin on CFTR Levels in Primary Human Airway Epithelial Cells Grown at the Air-Liquid-Interface.
Cystic Fibrosis
Effect of ATP concentration on CFTR Cl- channels: a kinetic analysis of channel regulation.
Cystic Fibrosis
Effect of ATP-sensitive K+ channel regulators on cystic fibrosis transmembrane conductance regulator chloride currents.
Cystic Fibrosis
Effect of cAMP agonists on cell pH and anion transport by cultured rat inner medullary collecting duct cells.
Cystic Fibrosis
Effect of cAMP on intracellular and extracellular ATP content of Cl- -secreting epithelia and 3T3 fibroblasts.
Cystic Fibrosis
Effect of CFTR correctors on the traffic and the function of intracellularly retained ABCB4 variants.
Cystic Fibrosis
Effect of CFTR Modulators on Anthropometric Parameters in Individuals with Cystic Fibrosis: An Evidence Analysis Center Systematic Review.
Cystic Fibrosis
Effect of chloride channel blockers on the cardiac CFTR chloride and L-type calcium currents.
Cystic Fibrosis
Effect of cystic fibrosis-associated mutations in the fourth intracellular loop of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Effect of cytosolic pH on epithelial Na+ channel in normal and cystic fibrosis sweat ducts.
Cystic Fibrosis
Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study.
Cystic Fibrosis
Effect of highly effective modulator therapy on quality of life in adults with cystic fibrosis.
Cystic Fibrosis
Effect of IBMX and alkaline phosphatase inhibitors on Cl- secretion in G551D cystic fibrosis mutant mice.
Cystic Fibrosis
Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR.
Cystic Fibrosis
Effect of ivacaftor therapy on exhaled nitric oxide in patients with cystic fibrosis.
Cystic Fibrosis
Effect of L-ascorbate on chloride transport in freshly excised sinonasal epithelia.
Cystic Fibrosis
Effect of lentivirus-mediated CFTR overexpression on oxidative stress injury and inflammatory response in the lung tissue of COPD mouse model.
Cystic Fibrosis
Effect of phenol red and steroid hormones on cystic fibrosis transmembrane conductance regulator in mouse endometrial epithelial cells.
Cystic Fibrosis
Effect of salinity and temperature on the expression of genes involved in branchial ion transport processes in European sea bass.
Cystic Fibrosis
Effect of the E4 region on the persistence of transgene expression from adenovirus vectors.
Cystic Fibrosis
Effect of the liquorice root derivatives on salt and water balance in a teleost fish, rainbow trout (Oncorhynchus mykiss).
Cystic Fibrosis
Effect of TongXie-YaoFang on Cl(-) and HCO3 (-) Transport in Diarrhea-Predominant Irritable Bowel Syndrome Rats.
Cystic Fibrosis
Effect of topiramate on sweat chloride level while screening for cystic fibrosis.
Cystic Fibrosis
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
Cystic Fibrosis
Effective silencing of ENaC by siRNA delivered with epithelial-targeted nanocomplexes in human cystic fibrosis cells and in mouse lung.
Cystic Fibrosis
Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations.
Cystic Fibrosis
Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial.
Cystic Fibrosis
Effects and interaction of dietary electrolyte balance and citric acid on the intestinal function of weaned piglets.
Cystic Fibrosis
Effects of a new cystic fibrosis transmembrane conductance regulator inhibitor on Cl- conductance in human sweat ducts.
Cystic Fibrosis
Effects of antiallergic herbal agents on cystic fibrosis transmembrane conductance regulator in nasal mucosal epithelia of allergic rhinitis rabbits.
Cystic Fibrosis
Effects of Azithromycin (AZM) on Glutathione-S-Transferases (GST)s in Cystic Fibrosis Airway Cells.
Cystic Fibrosis
Effects of C-terminal deletions on cystic fibrosis transmembrane conductance regulator function in cystic fibrosis airway epithelia.
Cystic Fibrosis
Effects of CFTR gene silencing by siRNA or the luminal application of a CFTR activator on fluid secretion from guinea-pig pancreatic duct cells.
Cystic Fibrosis
Effects of CFTR modulators on pharmacokinetics of tobramycin during acute pulmonary exacerbations in the pediatric cystic fibrosis population.
Cystic Fibrosis
Effects of chlorothalonil, prochloraz and the combination on intestinal barrier function and glucolipid metabolism in the liver of mice.
Cystic Fibrosis
Effects of Cordyceps sinensis, Cordyceps militaris and their isolated compounds on ion transport in Calu-3 human airway epithelial cells.
Cystic Fibrosis
Effects of cystic fibrosis transmembrane conductance regulator and DeltaF508CFTR on inflammatory response, ER stress, and Ca2+ of airway epithelia.
Cystic Fibrosis
Effects of early and late pneumothorax drainage on the development of pulmonary oedema.
Cystic Fibrosis
Effects of indacaterol on the LPS-evoked changes in fluid secretion rate and pH in swine tracheal membrane.
Cystic Fibrosis
Effects of Long-Term Exercise on Liver Cyst in Polycystic Liver Disease Model Rats.
Cystic Fibrosis
Effects of lovastatin on trafficking of cystic fibrosis transmembrane conductance regulator in human tracheal epithelium.
Cystic Fibrosis
Effects of low salinity media on growth, condition, and gill ion transporter expression in juvenile Gulf killifish, Fundulus grandis.
Cystic Fibrosis
Effects of membrane potential versus pHi on the cellular retention of doxorubicin analyzed via a comparison between cystic fibrosis transmembrane conductance regulator (CFTR) and multidrug resistance (MDR) transfectants.
Cystic Fibrosis
Effects of methacholine and uridine 5'-triphosphate on tracheal mucus rheology in mice.
Cystic Fibrosis
Effects of mutations in cAMP-dependent protein kinase on chloride efflux in Caco-2 human colonic carcinoma cells.
Cystic Fibrosis
Effects of nicorandil on the cAMP-dependent Cl- current in guinea-pig ventricular cells.
Cystic Fibrosis
Effects of P-glycoprotein expression on cyclic AMP and volume-activated ion fluxes and conductances in HT-29 colon adenocarcinoma cells.
Cystic Fibrosis
Effects of potassium ion supplementation on survival and ion regulation in Gulf killifish Fundulus grandis larvae reared in ion deficient saline waters.
Cystic Fibrosis
Effects of protein phosphatase and kinase inhibitors on Ca2+ and Cl- currents in guinea pig ventricular myocytes.
Cystic Fibrosis
Effects of purinergic stimulation, CFTR and osmotic stress on amiloride-sensitive Na+ transport in epithelia and Xenopus oocytes.
Cystic Fibrosis
Effects of pyrophosphate and nucleotide analogs suggest a role for ATP hydrolysis in cystic fibrosis transmembrane regulator channel gating.
Cystic Fibrosis
Effects of rhinovirus infection on the expression and function of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel in human nasal mucosa.
Cystic Fibrosis
Effects of salinity and prolactin on gene transcript levels of ion transporters, ion pumps and prolactin receptors in Mozambique tilapia intestine.
Cystic Fibrosis
Effects of sevoflurane on the cAMP-induced short-circuit current in mouse tracheal epithelium and recombinant Cl- (CFTR) and K+ (KCNQ1) channels.
Cystic Fibrosis
Effects of Slc26a6 deletion and CFTR inhibition on HCO3- secretion by mouse pancreatic duct.
Cystic Fibrosis
Effects of the delta F508 mutation on the structure, function, and folding of the first nucleotide-binding domain of CFTR.
Cystic Fibrosis
Efficacy and Safety of CFTR Corrector and Potentiator Combination Therapy in Patients with Cystic Fibrosis for the F508del-CFTR Homozygous Mutation: A Systematic Review and Meta-analysis.
Cystic Fibrosis
Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation.
Cystic Fibrosis
Efficacy and Safety of the CFTR Potentiator Icenticaftor (QBW251) in COPD: Results from a Phase 2 Randomized Trial.
Cystic Fibrosis
Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial.
Cystic Fibrosis
Efficacy of lumacaftor-ivacaftor for the treatment of cystic fibrosis patients homozygous for the F508del-CFTR mutation.
Cystic Fibrosis
Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis.
Cystic Fibrosis
Efficiency of plasmid delivery and expression after lipid-mediated gene transfer to human cells in vitro.
Cystic Fibrosis
Efficient 12-mutation testing in the CFTR gene: a general model for complex mutation analysis.
Cystic Fibrosis
Efficient Activation of Pathogenic ?Phe501 Mutation in Monocarboxylate Transporter 8 by Chemical and Pharmacological Chaperones.
Cystic Fibrosis
Efficient endocytosis of the cystic fibrosis transmembrane conductance regulator requires a tyrosine-based signal.
Cystic Fibrosis
Efficient gene transfer into myocardium by direct injection of adenovirus vectors.
Cystic Fibrosis
Efficient intracellular processing of the endogenous cystic fibrosis transmembrane conductance regulator in epithelial cell lines.
Cystic Fibrosis
Efficient intratracheal delivery of airway epithelial cells in mice and pigs.
Cystic Fibrosis
Efficient killing of inhaled bacteria in DeltaF508 mice: role of airway surface liquid composition.
Cystic Fibrosis
EG-VEGF, BV8, and their receptor expression in human bronchi and their modification in cystic fibrosis: Impact of CFTR mutation (delF508).
Cystic Fibrosis
Electrochemical immunosensors for the detection of survival motor neuron (SMN) protein using different carbon nanomaterials-modified electrodes.
Cystic Fibrosis
Electrodiffusional ATP movement through the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Electrogenic Na?/HCO?? co-transporter-1 is essential for the parathyroid hormone-stimulated intestinal HCO?? secretion.
Cystic Fibrosis
Electrolyte transport in the mammalian colon: mechanisms and implications for disease.
Cystic Fibrosis
Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host defense.
Cystic Fibrosis
Electronic health record phenotypes associated with genetically regulated expression of CFTR and application to cystic fibrosis.
Cystic Fibrosis
Electrophysiological characterization of chloride secretion across the jejunum and colon of pigs as affected by age and weaning.
Cystic Fibrosis
Electrophysiological evidence for the presence of cystic fibrosis transmembrane conductance regulator (CFTR) in mouse sperm.
Cystic Fibrosis
Electrostatic Tuning of Anion Attraction from the Cytoplasm to the Pore of the CFTR Chloride Channel.
Cystic Fibrosis
Elevated hepatic SULT1E1 activity in mouse models of cystic fibrosis alters the regulation of estrogen responsive proteins.
Cystic Fibrosis
Elevated paracellular glucose flux across cystic fibrosis airway epithelial monolayers is an important factor for Pseudomonas aeruginosa growth.
Cystic Fibrosis
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Cystic Fibrosis
Elexacaftor-Tezacaftor-Ivacaftor: The First Triple-Combination Cystic Fibrosis Transmembrane Conductance Regulator Modulating Therapy.
Cystic Fibrosis
Elexacaftor-tezacaftor-ivacaftor: The new paradigm to treat people with cystic fibrosis with at least one p.Phe508del mutation.
Cystic Fibrosis
Embryology of the Absent Vas Supported by 2 Cases of Congenital Unilateral Absence of Vas With Varied Associations.
Cystic Fibrosis
Emerging Cystic Fibrosis Transmembrane Conductance Regulator Modulators as New Drugs for Cystic Fibrosis: A Portrait of in Vitro Pharmacology and Clinical Translation.
Cystic Fibrosis
Emerging role of AMP-activated protein kinase in coupling membrane transport to cellular metabolism.
Cystic Fibrosis
Emerging role of CFTR as an epigenetic regulator - linking environmental cues to microRNAs.
Cystic Fibrosis
Emerging role of cystic fibrosis transmembrane conductance regulator - an epithelial chloride channel in gastrointestinal cancers.
Cystic Fibrosis
Emerging Technologies for Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Restoration in all People with CF.
Cystic Fibrosis
ENaC inhibition in cystic fibrosis: potential role in the new era of CFTR modulator therapies.
Cystic Fibrosis
ENaC regulation by phospholipids and DGK explained through mathematical modeling.
Cystic Fibrosis
ENaC- and CFTR-dependent ion and fluid transport in human middle ear epithelial cells.
Cystic Fibrosis
ENaC-mediated sodium influx exacerbates NLRP3-dependent inflammation in Cystic Fibrosis.
Cystic Fibrosis
Enamel pathology resulting from loss of function in the cystic fibrosis transmembrane conductance regulator in a porcine animal model.
Cystic Fibrosis
Endocervical metaplasia of the endometrium in a patient with cystic fibrosis: a case report.
Cystic Fibrosis
Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Endogenous ATP release inhibits electrogenic Na(+) absorption and stimulates Cl (-) secretion in MDCK cells.
Cystic Fibrosis
Endogenous concentrations of ouabain act as a cofactor to stimulate fluid secretion and cyst growth of in vitro ADPKD models via cAMP and EGFR-Src-MEK pathways.
Cystic Fibrosis
Endogenous expression of type II cGMP-dependent protein kinase mRNA and protein in rat intestine. Implications for cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Endogenous luminal surface adenosine signaling regulates duodenal bicarbonate secretion in rats.
Cystic Fibrosis
Endogenous or overexpressed cGMP-dependent protein kinases inhibit cAMP-dependent renin release from rat isolated perfused kidney, microdissected glomeruli, and isolated juxtaglomerular cells.
Cystic Fibrosis
Endogenous surface expression of ?F508-CFTR mediates cAMP-stimulated Cl(-) current in CFTR(?F508/?F508) pig thyroid epithelial cells.
Cystic Fibrosis
Endoplasmic reticulum stress and the unfolded protein response regulate genomic cystic fibrosis transmembrane conductance regulator expression.
Cystic Fibrosis
Endosomal SNARE proteins regulate CFTR activity and trafficking in epithelial cells.
Cystic Fibrosis
Energy Balance and Mechanisms of Weight Gain with Ivacaftor Treatment of Cystic Fibrosis Gating Mutations.
Cystic Fibrosis
Engraftment of donor-derived epithelial cells in multiple organs following bone marrow transplantation into newborn mice.
Cystic Fibrosis
Enhanced cell-surface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones.
Cystic Fibrosis
Enhanced delivery of peptide-morpholino oligonucleotides with a small molecule to correct splicing defects in the lung.
Cystic Fibrosis
Enhanced expression of ncc1 and clc2c in the kidney and urinary bladder accompanies freshwater acclimation in Mozambique tilapia.
Cystic Fibrosis
Enhancement by baclofen of the Gs-coupled receptor-mediated cAMP production in Xenopus oocytes expressing rat brain cortex poly (A)+ RNA: a role of G-protein beta gamma subunits.
Cystic Fibrosis
ENHANCEMENT OF ALVEOLAR EPITHELIAL SODIUM CHANNEL ACTIVITY WITH DECREASED CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR EXPRESSION IN MOUSE LUNG.
Cystic Fibrosis
Enhancement of ciliary beat amplitude by carbocisteine in ciliated human nasal epithelial cells.
Cystic Fibrosis
Enhancement of liposome-mediated gene transfer to human airway epithelial cells by replication-deficient adenovirus.
Cystic Fibrosis
Enhancing the efficiency of introducing precise mutations into the mouse genome by hit and run gene targeting.
Cystic Fibrosis
Enhancing the Potency of F508del Correction: A Multi-Layer Combinational Approach to Drug Discovery for Cystic Fibrosis.
Cystic Fibrosis
Enteric oxalate secretion is not directly mediated by the human CFTR chloride channel.
Cystic Fibrosis
Enterohemorrhagic E. coli (EHEC)-Secreted Serine Protease EspP Stimulates Electrogenic Ion Transport in Human Colonoid Monolayers.
Cystic Fibrosis
Enteroinvasive bacteria alter barrier and transport properties of human intestinal epithelium: role of iNOS and COX-2.
Cystic Fibrosis
Enzymatic and chemical probing of an S1 nuclease-sensitive site upstream from the human CFTR gene.
Cystic Fibrosis
EPAC1 activation by cAMP stabilizes CFTR at the membrane by promoting its interaction with NHERF1.
Cystic Fibrosis
Epidemiology and a novel procedure for large scale analysis of CFTR rearrangements in classic and atypical CF patients: a multicentric Italian study.
Cystic Fibrosis
Epidemiology and genetics of cystic fibrosis in Asia: In preparation for the next-generation treatments.
Cystic Fibrosis
Epidermal growth factor receptor activity upregulates lactate dehydrogenase A expression, lactate dehydrogenase activity, and lactate secretion in cultured IB3-1 cystic fibrosis lung epithelial cells.
Cystic Fibrosis
Epinephrine Stimulation of Anion Secretion in the Calu-3 Serous Cell Model.
Cystic Fibrosis
Episomal expression of wild-type CFTR corrects cAMP-dependent chloride transport in respiratory epithelial cells.
Cystic Fibrosis
Epithelial Barrier Properties of the 16HBE14o- Human Bronchial Epithelial Cell Culture Model.
Cystic Fibrosis
Epithelial cell contact-induced alterations in Salmonella enterica serovar Typhi lipopolysaccharide are critical for bacterial internalization.
Cystic Fibrosis
Epithelial IgG and its relationship to the loss of F508 in the common mutant form of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Epithelial ion channels in the regulation of female reproductive tract fluid microenvironment: implications in fertility and infertility.
Cystic Fibrosis
Epithelial mesenchymal transition (EMT): a universal process in lung diseases with implications for cystic fibrosis pathophysiology.
Cystic Fibrosis
Epithelial sodium channel inhibition in primary human bronchial epithelia by transfected siRNA.
Cystic Fibrosis
Epithelial sodium channel silencing as a strategy to correct the airway surface fluid deficit in cystic fibrosis.
Cystic Fibrosis
Epithelial sodium channels (ENaC) are uniformly distributed on motile cilia in the oviduct and the respiratory airways.
Cystic Fibrosis
Epithelial sodium channels regulate cystic fibrosis transmembrane conductance regulator chloride channels in Xenopus oocytes.
Cystic Fibrosis
Epitope conservation and immunohistochemical localization of the guanylin/stable toxin peptide receptor, guanylyl cyclase C.
Cystic Fibrosis
Epoxide-Mediated CifR Repression of cif Gene Expression Utilizes Two Binding Sites in Pseudomonas aeruginosa.
Cystic Fibrosis
ER-associated complexes (ERACs) containing aggregated cystic fibrosis transmembrane conductance regulator (CFTR) are degraded by autophagy.
Cystic Fibrosis
ERp29 regulates DeltaF508 and wild-type cystic fibrosis transmembrane conductance regulator (CFTR) trafficking to the plasma membrane in cystic fibrosis (CF) and non-CF epithelial cells.
Cystic Fibrosis
ERp29 Regulates Epithelial Sodium Channel Functional Expression by Promoting Channel Cleavage.
Cystic Fibrosis
Erratum to: A Stable Human-Cell System Overexpressing Cystic Fibrosis Transmembrane Conductance Regulator Recombinant Protein at the Cell Surface.
Cystic Fibrosis
Erythrocyte membrane ATP binding cassette (ABC) proteins: MRP1 and CFTR as well as CD39 (ecto-apyrase) involved in RBC ATP transport and elevated blood plasma ATP of cystic fibrosis.
Cystic Fibrosis
Escherichia coli-induced epithelial hyporesponsiveness to secretagogues is associated with altered CFTR localization.
Cystic Fibrosis
Essential fatty acid deficiency in relation to genotype in patients with cystic fibrosis.
Cystic Fibrosis
Establishment and characterization of a novel polarized MDCK epithelial cellular model for CFTR studies.
Cystic Fibrosis
Establishment and long-term culture of human cystic fibrosis endothelial cells.
Cystic Fibrosis
Estimating the age of p.(Phe508del) with family studies of geographically distinct European populations and the early spread of cystic fibrosis.
Cystic Fibrosis
Estrogen and progesterone differentially regulate the levels of cystic fibrosis transmembrane regulator (CFTR), adenylate cyclase (AC), and cyclic adenosine mono-phosphate (cAMP) in the rat cervix.
Cystic Fibrosis
Estrogen regulation of ion transporter messenger RNA levels in mouse efferent ductules are mediated differentially through estrogen receptor (ER) alpha and ER beta.
Cystic Fibrosis
Estrogen upregulates the expression levels and functional activities of duodenal mucosal CFTR and SLC26A6.
Cystic Fibrosis
Estrogen-dependent expression of the cystic fibrosis transmembrane regulator gene in a novel uterine epithelial cell line.
Cystic Fibrosis
Estrogen-induced abnormally high cystic fibrosis transmembrane conductance regulator expression results in ovarian hyperstimulation syndrome.
Cystic Fibrosis
Ethanol and its non-oxidative metabolites profoundly inhibit CFTR function in pancreatic epithelial cells which is prevented by ATP supplementation.
Cystic Fibrosis
Ethical dilemma: ELX/TEZ/IVA or Lung Transplantation in Cystic Fibrosis and End Stage Lung Disease?
Cystic Fibrosis
Ethnic heterogeneity and cystic fibrosis transmembrane regulator (CFTR) mutation frequencies in Chicago-area CF families.
Cystic Fibrosis
European Academy of Andrology guideline Management of oligo-astheno-teratozoospermia.
Cystic Fibrosis
Evaluation in vitro and in vivo of cationic liposome-expression construct complexes for cystic fibrosis gene therapy.
Cystic Fibrosis
Evaluation of 1,2,3-Triazoles as Amide Bioisosteres In Cystic Fibrosis Transmembrane Conductance Regulator Modulators VX-770 and VX-809.
Cystic Fibrosis
Evaluation of a BeadXpress Assay for a 151-Mutation and Variant CFTR Screening Panel After 11,000 Samples: Implications for Practice.
Cystic Fibrosis
Evaluation of autophagy inducers in epithelial cells carrying the ?F508 mutation of the cystic fibrosis transmembrane conductance regulator CFTR.
Cystic Fibrosis
Evaluation of gene targeting by homologous recombination in ovine somatic cells.
Cystic Fibrosis
Evaluation of high-resolution melting (HRM) for mutation scanning of selected exons of the CFTR gene.
Cystic Fibrosis
Evaluation of MRP1-5 gene expression in cystic fibrosis patients homozygous for the delta F508 mutation.
Cystic Fibrosis
Evaluation of potential regulatory elements identified as DNase I hypersensitive sites in the CFTR gene.
Cystic Fibrosis
Evaluation of repeat administration of a replication deficient, recombinant adenovirus containing the normal cystic fibrosis transmembrane conductance regulator cDNA to the airways of individuals with cystic fibrosis.
Cystic Fibrosis
Evaluation of the efficacy and safety of in vitro, adenovirus-mediated transfer of the human cystic fibrosis transmembrane conductance regulator cDNA.
Cystic Fibrosis
Evidence for a role of arginine vasotocin receptors in the gill during salinity acclimation by a euryhaline teleost fish.
Cystic Fibrosis
Evidence for alteration of calpain/calpastatin system in PBMC of cystic fibrosis patients.
Cystic Fibrosis
Evidence for an apical Na-Cl cotransporter involved in ion uptake in a teleost fish.
Cystic Fibrosis
Evidence for cystic fibrosis transmembrane conductance regulator chloride current in swine ventricular myocytes.
Cystic Fibrosis
Evidence for direct interaction between actin and the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Evidence for functional atypical nicotinic receptors that activate K+ dependent Cl- secretion in mouse tracheal epithelium.
Cystic Fibrosis
Evidence for independent Cl- and HCO3- secretion and involvement of an apical Na(+)-HCO3- cotransporter in cultured rat epididymal epithelia.
Cystic Fibrosis
Evidence for location of the CFTR in human placental apical membrane vesicles.
Cystic Fibrosis
Evidence for phosphorylation of serine 753 in CFTR using a novel metal-ion affinity resin and matrix-assisted laser desorption mass spectrometry.
Cystic Fibrosis
Evidence for safety and efficacy of DOTAP cationic liposome mediated CFTR gene transfer to the nasal epithelium of patients with cystic fibrosis.
Cystic Fibrosis
Evidence for the Involvement of Lipid Rafts and Plasma Membrane Sphingolipid Hydrolases in
Cystic Fibrosis
Evidence of a functional CFTR Cl(-) channel in adult alveolar epithelial cells.
Cystic Fibrosis
Evidence of Absorptive Function in vivo in a Neo-Formed Bio-Artificial Intestinal Segment Using a Rodent Model.
Cystic Fibrosis
Evidence that endoplasmic reticulum (ER)-associated degradation of cystic fibrosis transmembrane conductance regulator is linked to retrograde translocation from the ER membrane.
Cystic Fibrosis
Evidence that extracellular anions interact with a site outside the CFTR chloride channel pore to modify channel properties.
Cystic Fibrosis
Evidence that hereditary pancreatitis is genetically heterogeneous disorder.
Cystic Fibrosis
Evidence that release of adenosine triphosphate from endothelial cells during increased shear stress is vesicular.
Cystic Fibrosis
Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis.
Cystic Fibrosis
Evolutionary and functional divergence between the cystic fibrosis transmembrane conductance regulator and related ATP-binding cassette transporters.
Cystic Fibrosis
Evolving Mechanistic Views and Emerging Therapeutic Strategies for Cystic Fibrosis-Related Diabetes.
Cystic Fibrosis
Examination of potential overlap in autism and language loci on chromosomes 2, 7, and 13 in two independent samples ascertained for specific language impairment.
Cystic Fibrosis
Examining basal chloride transport using the nasal potential difference response in a murine model.
Cystic Fibrosis
Excess fluoride interferes with chloride-channel-dependent endocytosis in ameloblasts.
Cystic Fibrosis
Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa.
Cystic Fibrosis
Exocytosis is not involved in activation of Cl- secretion via CFTR in Calu-3 airway epithelial cells.
Cystic Fibrosis
Exon 9 of the CFTR gene: splice site haplotypes and cystic fibrosis mutations.
Cystic Fibrosis
Exon skipping through the creation of a putative exonic splicing silencer as a consequence of the cystic fibrosis mutation R553X.
Cystic Fibrosis
Exophiala dermatitidis Revealing Cystic Fibrosis in Adult Patients with Chronic Pulmonary Disease.
Cystic Fibrosis
Experimental assessment of splicing variants using expression minigenes and comparison with in silico predictions.
Cystic Fibrosis
Exposure of Pseudomonas aeruginosa to bactericidal hypochlorous acid during neutrophil phagocytosis is compromised in cystic fibrosis.
Cystic Fibrosis
Exposure to cigarette smoke condensate reduces calcium activated chloride channel transport in primary sinonasal epithelial cultures.
Cystic Fibrosis
Exposure to sodium butyrate leads to functional downregulation of calcium-activated potassium channels in human airway epithelial cells.
Cystic Fibrosis
Expression and characterization of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Expression and chromosome localization of the murine cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Expression and degradation of the cystic fibrosis transmembrane conductance regulator in Saccharomyces cerevisiae.
Cystic Fibrosis
Expression and Distribution of Cystic Fibrosis Transmembrane Conductance Regulator in Neurons of the Human Brain.
Cystic Fibrosis
Expression and distribution of cystic fibrosis transmembrane conductance regulator in neurons of the spinal cord.
Cystic Fibrosis
Expression and distribution of the Na(+)-HCO(-)(3) cotransporter in human pancreas.
Cystic Fibrosis
Expression and function of Anoctamin 1/TMEM16A calcium-activated chloride channels in airways of in vivo mouse models for cystic fibrosis research.
Cystic Fibrosis
Expression and function of CLC and cystic fibrosis transmembrane conductance regulator chloride channels in renal epithelial tubule cells: pathophysiological implications.
Cystic Fibrosis
Expression and function of cystic fibrosis transmembrane conductance regulator in rat intrapulmonary arteries.
Cystic Fibrosis
Expression and functional properties of the second predicted nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator fused to glutathione-S-transferase.
Cystic Fibrosis
Expression and intracellular processing of chimeric and mutant CFTR molecules.
Cystic Fibrosis
Expression and localization of cystic fibrosis transmembrane conductance regulator in human gingiva.
Cystic Fibrosis
Expression and localization of cystic fibrosis transmembrane conductance regulator in the rat endocrine pancreas.
Cystic Fibrosis
Expression and localization of the cystic fibrosis transmembrane conductance regulator mRNA and its protein in rat brain.
Cystic Fibrosis
Expression and purification of the cystic fibrosis transmembrane conductance regulator protein in Saccharomyces cerevisiae.
Cystic Fibrosis
Expression and purification of the first nucleotide-binding domain and linker region of human multidrug resistance gene product: comparison of fusions to glutathione S-transferase, thioredoxin and maltose-binding protein.
Cystic Fibrosis
Expression and purification of two hydrophobic double-spanning membrane proteins derived from the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Expression and regulation of chloride channels in neonatal rat cardiomyocytes.
Cystic Fibrosis
Expression and regulation of the cystic fibrosis gene during rat liver regeneration.
Cystic Fibrosis
Expression and regulation of the Na+-K+-2Cl- cotransporter NKCC1 in the normal and CFTR-deficient murine colon.
Cystic Fibrosis
Expression in Escherichia coli of cytoplasmic portions of the cystic fibrosis transmembrane conductance regulator: apparent bacterial toxicity of peptides containing R-domain sequences.
Cystic Fibrosis
Expression of a truncated cystic fibrosis transmembrane conductance regulator with an AAV5-pseudotyped vector in primates.
Cystic Fibrosis
Expression of alpha v beta 5 integrin is necessary for efficient adenovirus-mediated gene transfer in the human airway.
Cystic Fibrosis
Expression of an abundant alternatively spliced form of the cystic fibrosis transmembrane conductance regulator (CFTR) gene is not associated with a cAMP-activated chloride conductance.
Cystic Fibrosis
Expression of an ATP-binding cassette transporter-encoding gene (YOR1) is required for oligomycin resistance in Saccharomyces cerevisiae.
Cystic Fibrosis
Expression of carbonic anhydrase, cystic fibrosis transmembrane regulator (CFTR) and V-H(+)-ATPase in the lancelet Branchiostoma lanceolatum (Pallas, 1774).
Cystic Fibrosis
Expression of CFTR and a cAMP-stimulated chloride secretory current in cultured human fetal alveolar epithelial cells.
Cystic Fibrosis
Expression of CFTR and Cl(-) conductances in cells of pulmonary neuroepithelial bodies.
Cystic Fibrosis
Expression of CFTR and presence of cAMP-mediated fluid secretion in human fetal lung.
Cystic Fibrosis
Expression of CFTR controls cAMP-dependent activation of epithelial K+ currents.
Cystic Fibrosis
Expression of CFTR from a ciliated cell-specific promoter is ineffective at correcting nasal potential difference in CF mice.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator alters the responses to hypotonic cell swelling and ATP of Chinese hamster ovary cells.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator during early human embryo development.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator in a model epithelium.
Cystic Fibrosis
Expression of Cystic Fibrosis Transmembrane Conductance Regulator in Ganglia of Human Gastrointestinal Tract.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator in ganglion cells of the hearts.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator in human endometrium.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator in human gallbladder epithelial cells.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator in liver tissue from patients with cystic fibrosis.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator in paracervical ganglia.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator in rat efferent duct epithelium.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator in rat ovary.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator in the human distal lung.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane conductance regulator in the skin of the toad, Bufo bufo and possible role for Cl- transport across the heterocellular epithelium.
Cystic Fibrosis
Expression of cystic fibrosis transmembrane regulator Cl- channels in heart.
Cystic Fibrosis
Expression of delta F508 cystic fibrosis transmembrane conductance regulator protein and related chloride transport properties in the gallbladder epithelium from cystic fibrosis patients.
Cystic Fibrosis
Expression of DeltaF508 CFTR in normal mouse lung after site-specific modification of CFTR sequences by SFHR.
Cystic Fibrosis
Expression of DeltaF508 Cystic Fibrosis Transmembrane Regulator (CFTR) Decreases Membrane Sialylation.
Cystic Fibrosis
Expression of Drosophila melanogaster P-glycoproteins is associated with ATP channel activity.
Cystic Fibrosis
Expression of ENaC subunits, chloride channels, and aquaporins in ovine fetal lung: ontogeny of expression and effects of altered fetal cortisol concentrations.
Cystic Fibrosis
Expression of ion transport-associated proteins in human efferent and epididymal ducts.
Cystic Fibrosis
Expression of miR?542?3p in osteosarcoma with miRNA microarray data, and its potential signaling pathways.
Cystic Fibrosis
Expression of S100A8 correlates with inflammatory lung disease in congenic mice deficient of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Expression of SLC26A3, CFTR and NHE3 in the human male reproductive tract: role in male subfertility caused by congenital chloride diarrhoea.
Cystic Fibrosis
Expression of the chloride channel ClC-2 in the murine small intestine epithelium.
Cystic Fibrosis
Expression of the cystic fibrosis gene and the major pancreatic mucin gene, MUC1, in human ductal epithelial cells.
Cystic Fibrosis
Expression of the cystic fibrosis transmembrane conductance regulator (CFTR) mRNA in normal and pathological adult human epididymis.
Cystic Fibrosis
Expression of the cystic fibrosis transmembrane conductance regulator from a novel adeno-associated virus promoter.
Cystic Fibrosis
Expression of the cystic fibrosis transmembrane conductance regulator gene can be regulated by protein kinase C.
Cystic Fibrosis
Expression of the cystic fibrosis transmembrane conductance regulator gene in cells of non-epithelial origin.
Cystic Fibrosis
Expression of the cystic fibrosis transmembrane conductance regulator gene in the respiratory tract of normal individuals and individuals with cystic fibrosis.
Cystic Fibrosis
Expression of the cystic fibrosis transmembrane conductance regulator in rat spermatids: implication for the site of action of antispermatogenic agents.
Cystic Fibrosis
Expression of the human cystic fibrosis transmembrane conductance regulator gene in the mouse lung after in vivo intratracheal plasmid-mediated gene transfer.
Cystic Fibrosis
Expression of the receptor guanylyl cyclase C and its ligands in reproductive tissues of the rat: a potential role for a novel signaling pathway in the epididymis.
Cystic Fibrosis
Expression of voltage-gated potassium channels in human and mouse colonic carcinoma.
Cystic Fibrosis
Expression, localization, and binding activity of the ezrin/radixin/moesin proteins in the mouse testis.
Cystic Fibrosis
Expression, localization, and functional evaluation of CFTR in bovine corneal endothelial cells.
Cystic Fibrosis
Expressional Changes of Water Transport-related Molecules in the Efferent Ductules and Initial Segment of Mouse Treated with Bisphenol A-Containing Drinking Water for Two Generations.
Cystic Fibrosis
Extensive posttranscriptional deletion of the coding sequences for part of nucleotide-binding fold 1 in respiratory epithelial mRNA transcripts of the cystic fibrosis transmembrane conductance regulator gene is not associated with the clinical manifestations of cystic fibrosis.
Cystic Fibrosis
Extensive sequencing of the cystic fibrosis transmembrane regulator gene: assay validation and unexpected benefits of developing a comprehensive test.
Cystic Fibrosis
Extent of the selectivity filter conferred by the sixth transmembrane region in the CFTR chloride channel pore.
Cystic Fibrosis
External ATP and its analogs activate the cystic fibrosis transmembrane conductance regulator by a cyclic AMP-independent mechanism.
Cystic Fibrosis
External Zn(2+) binding to cysteine-substituted cystic fibrosis transmembrane conductance regulator constructs regulates channel gating and curcumin potentiation.
Cystic Fibrosis
Extracellular phosphate enhances the function of F508del-CFTR rescued by CFTR correctors.
Cystic Fibrosis
Extracellular UTP stimulates electrogenic bicarbonate secretion across CFTR knockout gallbladder epithelium.
Cystic Fibrosis
Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry.
Cystic Fibrosis
Extracts from peppermint leaves, lemon balm leaves and in particular angelica roots mimic the pro-secretory action of the herbal preparation STW 5 in the human intestine.
Cystic Fibrosis
Ezrin controls the macromolecular complexes formed between an adapter protein Na+/H+ exchanger regulatory factor and the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Ezrin links CFTR to TLR4 signaling to orchestrate anti-bacterial immune response in macrophages.
Cystic Fibrosis
Ezrin-radixin-moesin-binding phosphoprotein (EBP50), an estrogen-inducible scaffold protein, contributes to biliary epithelial cell proliferation.
Cystic Fibrosis
F508del CFTR gene mutation in patients with allergic bronchopulmonary aspergillosis.
Cystic Fibrosis
F508del disturbs the dynamics of the nucleotide binding domains of CFTR before and after ATP hydrolysis.
Cystic Fibrosis
Factors influencing readthrough therapy for frequent cystic fibrosis premature termination codons.
Cystic Fibrosis
Factors influencing the acquisition and eradication of early Pseudomonas aeruginosa infection in cystic fibrosis.
Cystic Fibrosis
Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers.
Cystic Fibrosis
Failure of the cystic fibrosis transmembrane conductance regulator to conduct ATP.
Cystic Fibrosis
False negative newborn screen and neonatal cholestasis in a premature child with cystic fibrosis.
Cystic Fibrosis
Familial concordance of phenotype and microbial variation among siblings with CF.
Cystic Fibrosis
Fas Expression in Conjunctival Epithelial Cells of Patients With Cystic Fibrosis.
Cystic Fibrosis
Feline immunodeficiency virus vectors persistently transduce nondividing airway epithelia and correct the cystic fibrosis defect.
Cystic Fibrosis
Female cystic fibrosis mutation carriers and assisted reproductive technology: does carrier status affect reproductive outcomes?
Cystic Fibrosis
Female gender hormones regulate mRNA levels and function of the rat lung epithelial Na channel.
Cystic Fibrosis
Fenretinide differentially modulates the levels of long- and very long-chain ceramides by downregulating Cers5 enzyme: evidence from bench to bedside.
Cystic Fibrosis
Fertility, Pregnancy and Lactation Considerations for Women with CF in the CFTR Modulator Era.
Cystic Fibrosis
Fetal Lung Epithelial Ion Channels Relocate in the Cell Membrane During Late Gestation.
Cystic Fibrosis
FFA3 Activation Stimulates Duodenal Bicarbonate Secretion and Prevents NSAID-Induced Enteropathy via the GLP-2 Pathway in Rats.
Cystic Fibrosis
First cloning and functional characterization of a melatonin receptor in fish brain: a novel one?
Cystic Fibrosis
First experience in Switzerland in Phe508del homozygous cystic fibrosis patients with end-stage pulmonary disease enrolled in a lumacaftor-ivacaftor therapy trial - preliminary results.
Cystic Fibrosis
First report of c. 1499G>C mutation in a 6-month-child with cystic fibrosis.
Cystic Fibrosis
First report of cystic fibrosis mutations in Libyan cystic fibrosis patients.
Cystic Fibrosis
First report of the cystic fibrosis transmembrane conductance regulator mutation c.1521_1523delCTT (p. Phe508del) in two Qatari patients with cystic fibrosis.
Cystic Fibrosis
Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis.
Cystic Fibrosis
FK506 binding protein 8 peptidylprolyl isomerase activity manages a late stage of cystic fibrosis transmembrane conductance regulator (CFTR) folding and stability.
Cystic Fibrosis
FKBP38 peptidylprolyl isomerase promotes the folding of cystic fibrosis transmembrane conductance regulator in the endoplasmic reticulum.
Cystic Fibrosis
FLAG epitope positioned in an external loop preserves normal biophysical properties of CFTR.
Cystic Fibrosis
Flagellin of Pseudomonas aeruginosa inhibits Na+ transport in airway epithelia.
Cystic Fibrosis
Flagellin-stimulated Cl- secretion and innate immune responses in airway epithelia: role for p38.
Cystic Fibrosis
Flickery block of single CFTR chloride channels by intracellular anions and osmolytes.
Cystic Fibrosis
Flos Magnoliae Inhibits Chloride Secretion via ANO1 Inhibition in Calu-3 Cells.
Cystic Fibrosis
Flow cytometry analysis reveals a decrease in intracellular sodium during sperm capacitation.
Cystic Fibrosis
Fluid secretion caused by aerolysin-like hemolysin of Aeromonas sobria in the intestines is due to stimulation of production of prostaglandin E2 via cyclooxygenase 2 by intestinal cells.
Cystic Fibrosis
Fluorescence assay for simultaneous quantification of CFTR ion-channel function and plasma membrane proximity.
Cystic Fibrosis
Fluorescence energy transfer detection as a homogeneous DNA diagnostic method.
Cystic Fibrosis
Fluorescence in situ hybridization mapping of the cystic fibrosis transmembrane conductance regulator (CFTR) gene to 7q31.3.
Cystic Fibrosis
Fluorescence-based oligonucleotide ligation assay for analysis of cystic fibrosis transmembrane conductance regulator gene mutations.
Cystic Fibrosis
Fluorescence-based, multiplex allele-specific PCR (MASPCR) detection of the delta F508 deletion in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
Fluorescent chloride indicators to assess the efficacy of CFTR cDNA delivery.
Cystic Fibrosis
Fluorescent multiplex microsatellites used to identify haplotype associations with 15 CFTR mutations in 124 Northern Irish CF families.
Cystic Fibrosis
Fluoride stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.
Cystic Fibrosis
Fluoxetine inhibits calcium-activated currents of salamander rod photoreceptor somata and presynaptic terminals via modulation of intracellular calcium dynamics.
Cystic Fibrosis
Focal adhesion kinase and osmotic responses in ionocytes of Fundulus heteroclitus, a euryhaline teleost fish.
Cystic Fibrosis
Folate Protects Hepatocytes of Hyperhomocysteinemia Mice From Apoptosis via Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Activated Endoplasmic Reticulum Stress.
Cystic Fibrosis
Folding and rescue of a cystic fibrosis transmembrane conductance regulator trafficking mutant identified using human-murine chimeric proteins.
Cystic Fibrosis
Fooling a freshwater fish: how dietary salt transforms the rainbow trout gill into a seawater gill phenotype.
Cystic Fibrosis
Forskolin and PMA pretreatment of HT29 cells alters their chloride conductance induced by cAMP, Ca2+ and hypotonic cell swelling.
Cystic Fibrosis
Forskolin-induced apical membrane insertion of virally expressed, epitope-tagged CFTR in polarized MDCK cells.
Cystic Fibrosis
Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients.
Cystic Fibrosis
Four adult patients with the missense mutation L206W and a mild cystic fibrosis phenotype.
Cystic Fibrosis
Four case reports of Chinese cystic fibrosis patients and literature review.
Cystic Fibrosis
Four new mutations of the CFTR gene (541delC, R347H, R352Q, E585X) detected by DGGE analysis in Italian CF patients, associated with different clinical phenotypes.
Cystic Fibrosis
Freedom of expression. Focus on "Endoplasmic reticulum stress and the unfolded protein response regulate cystic fibrosis transmembrane conductance regulator expression".
Cystic Fibrosis
Frequencies of cystic fibrosis mutations in the Maine population: high proportion of unknown alleles in individuals of French-Canadian ancestry.
Cystic Fibrosis
Frequency and clinical significance of the S1235R mutation in the cystic fibrosis transmembrane conductance regulator gene: results from a collaborative study.
Cystic Fibrosis
Frequency of CFTR gene mutations in males participating in an ICSI programme.
Cystic Fibrosis
Frequency of CFTR, SPINK1, and Cathepsin B Gene Mutation in North Indian Population: Connections between Genetics and Clinical Data.
Cystic Fibrosis
Frequency of cystic fibrosis transmembrane conductance regulator gene mutations and 5T allele in patients with allergic bronchopulmonary aspergillosis.
Cystic Fibrosis
Frequency of Cystic Fibrosis Transmembrane Conductance Regulator Variants in Individuals Evaluated for Primary Ciliary Dyskinesia.
Cystic Fibrosis
Frequency of delta F508 and haplotype association in Austrian cystic fibrosis families.
Cystic Fibrosis
Frequency of the delta F508 mutation and flanking marker haplotypes at the CF locus from 167 Czech families.
Cystic Fibrosis
Frequency of the F508 deletion in the CFTR gene in Turkish cystic fibrosis patients.
Cystic Fibrosis
Frequent occurrence of the CFTR intron 8 (TG)n 5T allele in men with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.
Cystic Fibrosis
Full-open and closed CFTR channels, with lateral tunnels from the cytoplasm and an alternative position of the F508 region, as revealed by molecular dynamics.
Cystic Fibrosis
Function and expression of cystic fibrosis transmembrane conductance regulator after small intestinal transplantation in mice.
Cystic Fibrosis
Function and regulation of TRPM7, as well as intracellular magnesium content, are altered in cells expressing ?F508-CFTR and G551D-CFTR.
Cystic Fibrosis
Function of the R domain in the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Function of the rat calcitonin receptors, C1a and C1b, expressed in Xenopus oocytes.
Cystic Fibrosis
Function of the second nucleotide-binding fold in the CFTR chloride channel.
Cystic Fibrosis
Function of Xenopus cystic fibrosis transmembrane conductance regulator (CFTR) Cl channels and use of human-Xenopus chimeras to investigate the pore properties of CFTR.
Cystic Fibrosis
Functional abnormalities in the retinal pigment epithelium of CFTR mutant mice.
Cystic Fibrosis
Functional Activity of Pat-1 (Slc26a6) Cl(-) /HCO(3) (-) Exchange in the Lower Villus Epithelium of Murine Duodenum.
Cystic Fibrosis
Functional analysis of a vacuolar ABC transporter in wild-type Candida albicans reveals its involvement in virulence.
Cystic Fibrosis
Functional analysis of cis-acting elements regulating the alternative splicing of human CFTR exon 9.
Cystic Fibrosis
Functional analysis of mutations in the putative binding site for cystic fibrosis transmembrane conductance regulator potentiators. Interaction between activation and inhibition.
Cystic Fibrosis
Functional analysis of nonsynonymous single nucleotide polymorphisms in human SLC26A9.
Cystic Fibrosis
Functional analysis of synonymous substitutions predicted to affect splicing of the CFTR gene.
Cystic Fibrosis
Functional analysis of the C-terminal boundary of the second nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator and structural implications.
Cystic Fibrosis
Functional and metabolic impairment in cigarette smoke-exposed macrophages is tied to oxidative stress.
Cystic Fibrosis
Functional and pharmacological induced structural changes of the cystic fibrosis transmembrane conductance regulator in the membrane solved using SAXS.
Cystic Fibrosis
Functional Architecture of the Cytoplasmic Entrance to the Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Pore.
Cystic Fibrosis
Functional arrangement of the 12th transmembrane region in the CFTR chloride channel pore based on functional investigation of a cysteine-less CFTR variant.
Cystic Fibrosis
Functional CFTR Expression in Cystic Fibrosis Airway Epithelial Cells by AAV6.2-mediated Segmental Trans-splicing.
Cystic Fibrosis
Functional Cftr in Crypt Epithelium of Organotypic Enteroid Cultures from Murine Small Intestine.
Cystic Fibrosis
Functional CFTR in endosomal compartment of CFTR-expressing fibroblasts and T84 cells.
Cystic Fibrosis
Functional characteristics of L1156F-CFTR associated with alcoholic chronic pancreatitis in Japanese.
Cystic Fibrosis
Functional characterization of a novel CFTR mutation P67S identified in a patient with atypical cystic fibrosis.
Cystic Fibrosis
Functional characterization of a recombinant adeno-associated virus 5-pseudotyped cystic fibrosis transmembrane conductance regulator vector.
Cystic Fibrosis
Functional characterization of cystic fibrosis transmembrane conductance regulator (CFTR) in apical membranes purified from bovine tracheal epithelium.
Cystic Fibrosis
Functional characterization of various channel-expressing central airway epithelial cells from mouse induced pluripotent stem cells.
Cystic Fibrosis
Functional characterization reveals that zebrafish CFTR prefers to occupy closed channel conformations.
Cystic Fibrosis
Functional classification of gill ionocytes and spatiotemporal changes in their distribution after transfer from seawater to freshwater in Japanese seabass.
Cystic Fibrosis
Functional classification of mitochondrion-rich cells in euryhaline Mozambique tilapia (Oreochromis mossambicus) embryos, by means of triple immunofluorescence staining for Na+/K+-ATPase, Na+/K+/2Cl- cotransporter and CFTR anion channel.
Cystic Fibrosis
Functional comparison of mouse slc26a6 anion exchanger with human SLC26A6 polypeptide variants: differences in anion selectivity, regulation, and electrogenicity.
Cystic Fibrosis
Functional consequences of heterologous expression of the cystic fibrosis transmembrane conductance regulator in fibroblasts.
Cystic Fibrosis
Functional coupling of Gs and CFTR is independent of their association with lipid rafts in epithelial cells.
Cystic Fibrosis
Functional cystic fibrosis transmembrane conductance regulator expression in cystic fibrosis airway epithelial cells by AAV6.2-mediated segmental trans-splicing.
Cystic Fibrosis
Functional cystic fibrosis transmembrane conductance regulator tagged with an epitope of the vesicular stomatis virus glycoprotein can be addressed to the apical domain of polarized cells.
Cystic Fibrosis
Functional defect of variants in the adenosine triphosphate-binding sites of ABCB4 and their rescue by the cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor (VX-770).
Cystic Fibrosis
Functional Differences in Pore Properties Between Wild-Type and Cysteine-Less Forms of the CFTR Chloride Channel.
Cystic Fibrosis
Functional dissection of the R domain of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Functional domain analysis of the yeast ABC transporter Ycf1p by site-directed mutagenesis.
Cystic Fibrosis
Functional evidence of CFTR gene transfer in nasal epithelium of cystic fibrosis mice in vivo following luminal application of DNA complexes targeted to the serpin-enzyme complex receptor.
Cystic Fibrosis
Functional expression and apical localization of the cystic fibrosis transmembrane conductance regulator in MDCK I cells.
Cystic Fibrosis
Functional Expression of a Ca(2+)-activated Cl(-) Channel Modulator Involved in Ion Transport and Epithelial Cell Differentiation.
Cystic Fibrosis
Functional expression of adrenergic and opioid receptors in Xenopus oocytes: interaction between alpha 2- and beta 2-adrenergic receptors.
Cystic Fibrosis
FUNCTIONAL EXPRESSION OF CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR IN MOUSE CHONDROCYTES.
Cystic Fibrosis
Functional expression of cystic fibrosis transmembrane conductance regulator in rat oviduct epithelium.
Cystic Fibrosis
Functional expression of G protein-coupled receptor 30 in immature rat epididymal epithelium.
Cystic Fibrosis
Functional expression of the cystic fibrosis transmembrane conductance regulator in yeast.
Cystic Fibrosis
Functional Gene Correction for Cystic Fibrosis in Lung Epithelial Cells Generated from Patient iPSCs.
Cystic Fibrosis
Functional genomic responses to cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR(delta508) in the lung.
Cystic Fibrosis
Functional human CFTR produced by stable Chinese hamster ovary cell lines derived using yeast artificial chromosomes.
Cystic Fibrosis
Functional IL-10 deficiency in the lung of cystic fibrosis (cftr(-/-)) and IL-10 knockout mice causes increased expression and function of B7 costimulatory molecules on alveolar macrophages.
Cystic Fibrosis
Functional integrity of the vesicle transporting machinery is required for complete activation of cFTR expressed in xenopus laevis oocytes.
Cystic Fibrosis
Functional interaction between CFTR and Cx45 gap junction channels expressed in oocytes.
Cystic Fibrosis
Functional interaction between CFTR and the sodium-phosphate co-transport type 2a in Xenopus laevis oocytes.
Cystic Fibrosis
Functional interaction between TRP4 and CFTR in mouse aorta endothelial cells.
Cystic Fibrosis
Functional interaction of the cystic fibrosis transmembrane conductance regulator with members of the SLC26 family of anion transporters (SLC26A8 and SLC26A9): Physiological and pathophysiological relevance.
Cystic Fibrosis
Functional interactions of HCO3- with cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Functional ion channels in pulmonary alveolar type I cells support a role for type I cells in lung ion transport.
Cystic Fibrosis
Functional modifications of Acid-sensing ion channels by ligand-gated chloride channels.
Cystic Fibrosis
Functional organization of cytoplasmic portals controlling access to the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel pore.
Cystic Fibrosis
Functional reconstitution and channel activity measurements of purified wildtype and mutant CFTR protein.
Cystic Fibrosis
Functional rescue of an ABCB11 mutant by ivacaftor: A new targeted pharmacotherapy approach in bile salt export pump deficiency.
Cystic Fibrosis
Functional roles of nonconserved structural segments in CFTR's NH2-terminal nucleotide binding domain.
Cystic Fibrosis
Functional roles of the nucleotide-binding folds in the activation of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Functional stability of CFTR depends on tight binding of ATP at its degenerate ATP-binding site.
Cystic Fibrosis
Functional Stability of Rescued {Delta}F508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Airway Epithelial Cells.
Cystic Fibrosis
Functional Vacuolar ATPase (V-ATPase) proton pumps traffic to the enterocyte brush border membrane and require CFTR.
Cystic Fibrosis
Functions of the cystic fibrosis transmembrane conductance regulator protein.
Cystic Fibrosis
G protein G alpha i-2 inhibits outwardly rectifying chloride channels in human airway epithelial cells.
Cystic Fibrosis
G-protein regulation of outwardly rectified epithelial chloride channels incorporated into planar bilayer membranes.
Cystic Fibrosis
G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects.
Cystic Fibrosis
G551D Mutation Impairs Protein Kinase A (PKA)-dependent Activation of CFTR Channel that can be Restored by Novel Gain-Of-Function (GOF) Mutations.
Cystic Fibrosis
G970R-CFTR Mutation (c.2908G>C) Results Predominantly in a Splicing Defect.
Cystic Fibrosis
GABA induces thee differentiation of small into large cholangiocytes by activation of Ca(2+) /CaMK I-dependent adenylyl cyclase 8.
Cystic Fibrosis
Gating of amiloride-sensitive Na(+) channels: subunit-subunit interactions and inhibition by the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Gating of cystic fibrosis transmembrane conductance regulator chloride channels by adenosine triphosphate hydrolysis. Quantitative analysis of a cyclic gating scheme.
Cystic Fibrosis
Gating of the CFTR Cl\#8722; channel by ATP-driven nucleotide-binding domain dimerisation.
Cystic Fibrosis
Gender-sensitive association of CFTR gene mutations and 5T allele emerging from a large survey on infertility.
Cystic Fibrosis
Gender-specific protection of estrogen against gastric acid-induced duodenal injury: stimulation of duodenal mucosal bicarbonate secretion.
Cystic Fibrosis
Gene expression after freshwater transfer in gills and opercular epithelia of killifish: insight into divergent mechanisms of ion transport.
Cystic Fibrosis
Gene SNPs and mutations in clinical genetic testing: haplotype-based testing and analysis.
Cystic Fibrosis
Gene therapy for cystic fibrosis airway disease- is clinical success imminent?
Cystic Fibrosis
Gene therapy for cystic fibrosis lung disease: current status and future perspectives.
Cystic Fibrosis
Gene Therapy for Cystic Fibrosis Paved the Way for the Use of Adeno-Associated Virus in Gene Therapy.
Cystic Fibrosis
Gene therapy for cystic fibrosis utilizing a replication deficient recombinant adenovirus vector to deliver the human cystic fibrosis transmembrane conductance regulator cDNA to the airways. A phase I study.
Cystic Fibrosis
Gene Therapy for Cystic Fibrosis: Progress and Challenges of Genome Editing.
Cystic Fibrosis
Gene therapy in a xenograft model of cystic fibrosis lung corrects chloride transport more effectively than the sodium defect.
Cystic Fibrosis
Gene transduction and cell entry pathway of fiber-modified adenovirus type 5 vectors carrying novel endocytic peptide ligands selected on human tracheal glandular cells.
Cystic Fibrosis
Gene transfer into the fetal primate: evidence for the secretion of transgene product.
Cystic Fibrosis
Gene transfer to freshly isolated human respiratory epithelial cells in vitro using a replication-deficient adenovirus containing the human cystic fibrosis transmembrane conductance regulator cDNA.
Cystic Fibrosis
Gene transfer to human fetal pulmonary tissue developed in immunodeficient SCID mice.
Cystic Fibrosis
Gene transfer to the tracheobronchial tree: implications for fetal gene therapy for cystic fibrosis.
Cystic Fibrosis
Gene-specific MicroRNA antagonism protects against HIV Tat and TGF-?-mediated suppression of CFTR mRNA and function.
Cystic Fibrosis
General anesthetic octanol and related compounds activate wild-type and delF508 cystic fibrosis chloride channels.
Cystic Fibrosis
Generation and phenotype of cell lines derived from CF and non-CF mice that carry the H-2K(b)-tsA58 transgene.
Cystic Fibrosis
Generation of a gene-corrected isogenic control iPSC line from cystic fibrosis patient-specific iPSCs homozygous for p.Phe508del mutation mediated by TALENs and ssODN.
Cystic Fibrosis
Generation of an induced pluripotent stem cell line (MHHi018-A) from a patient with Cystic Fibrosis carrying p.Asn1303Lys (N1303K) mutation.
Cystic Fibrosis
Generation of functional cholangiocyte-like cells from human pluripotent stem cells and HepaRG cells.
Cystic Fibrosis
Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study.
Cystic Fibrosis
Generation of Induced Progenitor-like Cells from Mature Epithelial Cells Using Interrupted Reprogramming.
Cystic Fibrosis
Generation of KCL021 research grade human embryonic stem cell line carrying a ?F508 mutation in the CFTR gene.
Cystic Fibrosis
Generation of KCL029 research grade human embryonic stem cell line carrying a mutation in WAS gene.
Cystic Fibrosis
Generation of multiciliated cells in functional airway epithelia from human induced pluripotent stem cells.
Cystic Fibrosis
Generation of Novel AAV Variants by Directed Evolution for Improved CFTR Delivery to Human Ciliated Airway Epithelium.
Cystic Fibrosis
Genes in the vicinity of CFTR modulate the cystic fibrosis phenotype in highly concordant or discordant F508del homozygous sib pairs.
Cystic Fibrosis
Genetic analysis of rwandan patients with cystic fibrosis-like symptoms: identification of novel cystic fibrosis transmembrane conductance regulator and epithelial sodium channel gene variants.
Cystic Fibrosis
Genetic analysis of the bicarbonate secreting anion exchanger SLC26A6 in chronic pancreatitis.
Cystic Fibrosis
Genetic and biochemical markers of obstructive lung disease in the general population.
Cystic Fibrosis
Genetic and clinical features of false-negative infants in a neonatal screening programme for cystic fibrosis.
Cystic Fibrosis
Genetic and phenotypic traits of children and adolescents with cystic fibrosis in Southern Brazil.
Cystic Fibrosis
Genetic basis of variable exon 9 skipping in cystic fibrosis transmembrane conductance regulator mRNA.
Cystic Fibrosis
Genetic characterisation of circulating fetal cells allows non-invasive prenatal diagnosis of cystic fibrosis.
Cystic Fibrosis
Genetic comparisons of patients with cystic fibrosis with or without meconium ileus. Clinical Centers of the French CF Registry.
Cystic Fibrosis
Genetic cystic fibrosis transmembrane regulator 4016insT D1152H compound heterozygosity and male infertility: an Italian case report.
Cystic Fibrosis
Genetic deletion of keratin 8 corrects the altered bone formation and osteopenia in a mouse model of cystic fibrosis.
Cystic Fibrosis
Genetic determinants and epidemiology of cystic fibrosis-related diabetes: results from a British cohort of children and adults.
Cystic Fibrosis
Genetic diagnosis in infertile men with numerical and constitutional sperm abnormalities.
Cystic Fibrosis
Genetic Evaluation of Children with Idiopathic Recurrent Acute Pancreatitis.
Cystic Fibrosis
Genetic factors in chronic pancreatitis; implications for diagnosis, management and prognosis.
Cystic Fibrosis
Genetic investigations of CFTR mutations in congenital absence of vas deferens, uterus, and vagina as a cause of infertility.
Cystic Fibrosis
Genetic markers of male infertility: Y chromosome microdeletions and cystic fibrosis transmembrane conductance gene mutations.
Cystic Fibrosis
Genetic modification of cystic fibrosis with ?F508 mutation of CFTR gene using the CRISPR system in peripheral blood mononuclear cells.
Cystic Fibrosis
Genetic risk factors in infertile men with severe oligozoospermia and azoospermia.
Cystic Fibrosis
Genetic screening in 2,710 infertile candidate couples for assisted reproductive techniques: results of application of Italian guidelines for the appropriate use of genetic tests.
Cystic Fibrosis
Genetic screening in Italian infertile couples undergoing intrauterine insemination and in vitro fertilization techniques: a multicentric study.
Cystic Fibrosis
Genetic variation within the ovine cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Genetic, andrological and clinical characteristics of patients with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
Genetic, cell biological, and clinical interrogation of the CFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention.
Cystic Fibrosis
Genetic, epidemiological, and clinical aspects of hereditary pancreatitis: a population-based cohort study in Denmark.
Cystic Fibrosis
Genetics of chronic obstructive pulmonary disease, beyond a1-antitrypsin deficiency.
Cystic Fibrosis
Genetics of chronic rhinosinusitis: State of the field and directions forward.
Cystic Fibrosis
Genetics of cystic fibrosis: CFTR mutation classifications toward genotype-based CF therapies.
Cystic Fibrosis
Genistein activates CFTR Cl- channels via a tyrosine kinase- and protein phosphatase-independent mechanism.
Cystic Fibrosis
Genistein activates CFTR-mediated Cl(-) secretion in the murine trachea and colon.
Cystic Fibrosis
Genistein and tyrphostin 47 stimulate CFTR-mediated Cl- secretion in T84 cell monolayers.
Cystic Fibrosis
Genistein directly induces cardiac CFTR chloride current by a tyrosine kinase-independent and protein kinase A-independent pathway in guinea pig ventricular myocytes.
Cystic Fibrosis
Genistein improves regulatory interactions between G551D-cystic fibrosis transmembrane conductance regulator and the epithelial sodium channel in Xenopus oocytes.
Cystic Fibrosis
Genistein modifies the activation kinetics and magnitude of phosphorylated wild-type and G551D-CFTR chloride currents.
Cystic Fibrosis
Genistein restores functional interactions between Delta F508-CFTR and ENaC in Xenopus oocytes.
Cystic Fibrosis
Genistein stimulates duodenal HCO(3)(-) secretion through PI3K pathway in mice.
Cystic Fibrosis
Genistein-Calcitriol Mitigates Hyperosmotic Stress-Induced TonEBP, CFTR Dysfunction, VDR Degradation and Inflammation in Dry Eye Disease.
Cystic Fibrosis
Genistein-induced fluid accumulation in ovariectomised rats' uteri is associated with increased cystic fibrosis transmembrane regulator expression.
Cystic Fibrosis
Genome-wide analysis of MicroRNA-messenger RNA interactome in ex-vivo gill filaments, Anguilla japonica.
Cystic Fibrosis
Genome-wide association study for poor sperm motility in Holstein-Friesian bulls.
Cystic Fibrosis
Genomewide association analysis of respiratory syncytial virus infection in mice.
Cystic Fibrosis
Genomic DNA sequence of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
Genomic rearrangements in the CFTR gene: extensive allelic heterogeneity and diverse mutational mechanisms.
Cystic Fibrosis
Genomic sequence analysis of Fugu rubripes CFTR and flanking genes in a 60 kb region conserving synteny with 800 kb of human chromosome 7.
Cystic Fibrosis
Genomic sequencing in cystic fibrosis newborn screening: what works best, two-tier predefined CFTR mutation panels or second-tier CFTR panel followed by third-tier sequencing?
Cystic Fibrosis
Genotype patterns for mutations of the cystic fibrosis transmembrane conductance regulator gene: a retrospective descriptive study from Saudi Arabia.
Cystic Fibrosis
Genotype-phenotype correlation and functional studies in patients with cystic fibrosis bearing CFTR complex alleles.
Cystic Fibrosis
Genotype-phenotype correlation in 9 patients with tropical pancreatitis and identified gene mutations.
Cystic Fibrosis
Genotype-phenotype correlations in cystic fibrosis: clinical severity of mutation S549R(T-->G).
Cystic Fibrosis
Genotype-phenotype relationship for five CFTR mutations frequently identified in western France.
Cystic Fibrosis
Genotype-phenotype relationships in a cohort of adult cystic fibrosis patients.
Cystic Fibrosis
Genotype/phenotype correlation of the G85E mutation in a large cohort of cystic fibrosis patients.
Cystic Fibrosis
Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis.
Cystic Fibrosis
Genotyping microarray for the detection of more than 200 CFTR mutations in ethnically diverse populations.
Cystic Fibrosis
Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations.
Cystic Fibrosis
Geographic distribution of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Saudi Arabia.
Cystic Fibrosis
Geographic distribution of cystic fibrosis transmembrane regulator gene mutations in Saudi Arabia.
Cystic Fibrosis
Geographic heterogeneity of 4 common worldwide cystic fibrosis non-DF508 mutations in Brazil.
Cystic Fibrosis
Germline mutations and gene polymorphism associated with human pancreatitis.
Cystic Fibrosis
Germline mutations in CFTR and PSTI genes in chronic pancreatitis patients.
Cystic Fibrosis
Gestational and tissue-specific regulation of C1C-2 chloride channel expression.
Cystic Fibrosis
GFP-tagged CFTR transgene is functional in the G551D cystic fibrosis mouse colon.
Cystic Fibrosis
Glandular Proteome Identifies Antiprotease Cystatin C as a Critical Modulator of Airway Hydration and Clearance.
Cystic Fibrosis
Glial cells express multiple ATP binding cassette proteins which are involved in ATP release.
Cystic Fibrosis
Glibenclamide induces apoptosis through inhibition of cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channels and intracellular Ca(2+) release in HepG2 human hepatoblastoma cells.
Cystic Fibrosis
Glibenclamide inhibits BK polyomavirus infection in kidney cells through CFTR blockade.
Cystic Fibrosis
Glibenclamide stimulates fluid secretion in rodent cholangiocytes through a cystic fibrosis transmembrane conductance regulator-independent mechanism.
Cystic Fibrosis
Glibenclamide, an ATP-sensitive K+ channel blocker, inhibits cardiac cAMP-activated Cl- conductance.
Cystic Fibrosis
Global assessment of the integrated stress response in CF patient-derived airway and intestinal tissues.
Cystic Fibrosis
Global proteomic approach unmasks involvement of keratins 8 and 18 in the delivery of cystic fibrosis transmembrane conductance regulator (CFTR)/deltaF508-CFTR to the plasma membrane.
Cystic Fibrosis
GLPG1837, a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1).
Cystic Fibrosis
Glucocorticoid dexamethasone regulates the differentiation of mouse conducting airway epithelial progenitor cells.
Cystic Fibrosis
Glucocorticoids and myosin5b loss of function induce heightened PKA signaling in addition to membrane traffic defects.
Cystic Fibrosis
Glucocorticoids Distinctively Modulate the CFTR Channel with Possible Implications in Lung Development and Transition into Extrauterine Life.
Cystic Fibrosis
Glucose homeostasis and genotype-phenotype interplay in cystic fibrosis patients with CFTR gene deltaF508 mutation.
Cystic Fibrosis
Glucose sensing mechanisms in hypothalamic cell models: glucose inhibition of AgRP synthesis and secretion.
Cystic Fibrosis
Glutamate acts at NMDA receptors on fresh bovine and on cultured human retinal pigment epithelial cells to trigger release of ATP.
Cystic Fibrosis
Glutathione levels and BAX activation during apoptosis due to oxidative stress in cells expressing wild-type and mutant cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.
Cystic Fibrosis
Glycosylation status of endogenous CFTR does not affect cAMP-stimulated Cl- secretion in epithelial cells.
Cystic Fibrosis
Gout-causing Q141K mutation in ABCG2 leads to instability of the nucleotide-binding domain and can be corrected with small molecules.
Cystic Fibrosis
Gp78 Cooperates with RMA1 in Endoplasmic Reticulum-associated Degradation of CFTR{Delta}F508.
Cystic Fibrosis
Gramicidin-perforated patch analysis on HCO3- secretion through a forskolin-activated anion channel in rat parotid intralobular duct cells.
Cystic Fibrosis
Green fluorescent protein-based halide indicators with improved chloride and iodide affinities.
Cystic Fibrosis
Growth and nutritional status in children and adolescents with cystic fibrosis.
Cystic Fibrosis
Growth deficits in cystic fibrosis mice begin in utero prior to IGF-1 reduction.
Cystic Fibrosis
Growth of putative progenitors of type II pneumocytes in culture of human cystic fibrosis alveoli.
Cystic Fibrosis
Guanylin activates Cl- secretion into the lumen of seawater eel intestine via apical Cl- channel under simulated in vivo conditions.
Cystic Fibrosis
Guanylin and functional coupling proteins in the human salivary glands and gland tumors : expression, cellular localization, and target membrane domains.
Cystic Fibrosis
Guanylin and uroguanylin in the parotid and submandibular glands: potential intrinsic regulators of electrolyte secretion in salivary glands.
Cystic Fibrosis
Guanylin in the human pancreas: a novel luminocrine regulatory pathway of electrolyte secretion via cGMP and CFTR in the ductal system.
Cystic Fibrosis
Guanylin peptides regulate electrolyte and fluid transport in the Gulf toadfish (Opsanus beta) posterior intestine.
Cystic Fibrosis
Guanylin regulates chloride secretion in the human gallbladder via the bile fluid.
Cystic Fibrosis
Guanylin strongly stimulates rat duodenal HCO3- secretion: proposed mechanism and comparison with other secretagogues.
Cystic Fibrosis
Guidelines for the clinical management and follow-up of infants with inconclusive cystic fibrosis diagnosis through newborn screening.
Cystic Fibrosis
H2O2 Stimulates Cystic Fibrosis Transmembrane Conductance Regulator through an Autocrine Prostaglandin Pathway, Using Multidrug-Resistant Protein-4.
Cystic Fibrosis
H5N1 Virus Haemagglutinin Inhibition of cAMP-Dependent CFTR via TLR4-Mediated Janus Tyrosine Kinase 3 Activation Exacerbates Lung Inflammation.
Cystic Fibrosis
Halide permeation in wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride channels.
Cystic Fibrosis
HALT-D: A Phase II Evaluation of Crofelemer for the Prevention and Prophylaxis of Diarrhea in Patients With Breast Cancer on Pertuzumab-Based Regimens.
Cystic Fibrosis
Harnessing Neutrophil Survival Mechanisms during Chronic Infection by Pseudomonas aeruginosa: Novel Therapeutic Targets to Dampen Inflammation in Cystic Fibrosis.
Cystic Fibrosis
HCO(3)(-)-dependent soluble adenylyl cyclase activates cystic fibrosis transmembrane conductance regulator in corneal endothelium.
Cystic Fibrosis
Head growth in cystic fibrosis following early diagnosis by neonatal screening.
Cystic Fibrosis
Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?
Cystic Fibrosis
Heat treatment of thioredoxin fusions increases the purity of ?-helical transmembrane protein constructs.
Cystic Fibrosis
Heat-stable enterotoxin of Escherichia coli (STa) can stimulate duodenal HCO3(-) secretion via a novel GC-C- and CFTR-independent pathway.
Cystic Fibrosis
HEK-293 cells expressing the cystic fibrosis transmembrane conductance regulator (CFTR): a model for studying regulation of Cl- transport.
Cystic Fibrosis
Helicobacter pylori infection downregulates duodenal CFTR and SLC26A6 expressions through TGF? signaling pathway.
Cystic Fibrosis
Hematopoietic Stem/Progenitor Cells Express Functional Mitochondrial Energy-Dependent Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
Herbal components of Japanese Kampo medicines exert laxative actions in colonic epithelium cells via activation of BK and CFTR channels.
Cystic Fibrosis
Hereditary pancreatitis in Japan: a review of pancreatitis-associated gene mutations.
Cystic Fibrosis
Heritability of Respiratory Infection with Pseudomonas aeruginosa in Cystic Fibrosis.
Cystic Fibrosis
Hesperidin stimulates cystic fibrosis transmembrane conductance regulator-mediated chloride secretion and ciliary beat frequency in sinonasal epithelium.
Cystic Fibrosis
Heterogeneity in mouse spasmolytic polypeptide-expressing metaplasia lineages identifies markers of metaplastic progression.
Cystic Fibrosis
Heterogeneity in the severity of cystic fibrosis and the role of CFTR gene mutations.
Cystic Fibrosis
Heterogeneity of phenotype in two cystic fibrosis patients homozygous for the CFTR exon 11 mutation G551D.
Cystic Fibrosis
Heterogeneity of reproductive tract abnormalities in men with absence of the vas deferens: role of cystic fibrosis transmembrane conductance regulator gene mutations.
Cystic Fibrosis
Heterogeneity of the cystic fibrosis phenotype in a large kindred family in Qatar with cystic fibrosis mutation (I1234V).
Cystic Fibrosis
Heterogeneous Spectrum of CFTR Gene Mutations in Korean Patients with Cystic Fibrosis.
Cystic Fibrosis
Heterogeneous spectrum of mutations in CFTR gene from Indian patients with congenital absence of the vas deferens and their association with cystic fibrosis genetic modifiers.
Cystic Fibrosis
Heterogenous spectrum of CFTR gene mutations in Indian patients with congenital absence of vas deferens.
Cystic Fibrosis
Heterologous expression of delta F508 CFTR results in decreased sialylation of membrane glycoconjugates.
Cystic Fibrosis
Heterologous expression systems for study of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Heterologous regulation of anion transporters by menthol in human airway epithelial cells.
Cystic Fibrosis
Heterozygosity for the F508del mutation in the cystic fibrosis transmembrane conductance regulator anion channel attenuates influenza severity.
Cystic Fibrosis
Heterozygous Cystic Fibrosis Transmembrane Regulator Gene Missense Variants Are Associated With Worse Cardiac Function in Patients With Duchenne Muscular Dystrophy.
Cystic Fibrosis
Heterozygous variant at nucleotide position 875+11A>T in exon 6A cystic fibrosis transmembrane conductance regulator gene induces 852del22 mutation false-positivity by line probe assay.
Cystic Fibrosis
High allelic heterogeneity between Afro-Brazilians and Euro-Brazilians impacts cystic fibrosis genetic testing.
Cystic Fibrosis
High CFTR expression in Philadelphia chromosome-positive acute leukemia protects and maintains continuous activation of BCR-ABL and related signaling pathways in combination with PP2A.
Cystic Fibrosis
High density lipoprotein promoting proliferation and migration of type II alveolar epithelial cells during inflammation state.
Cystic Fibrosis
High frequency of (TG)mTn variant tracts in the cystic fibrosis transmembrane conductance regulator gene in men with high semen viscosity.
Cystic Fibrosis
High frequency of cystic fibrosis transmembrane regulator mutation L997F in patients with recurrent idiopathic pancreatitis and in newborns with hypertrypsinemia.
Cystic Fibrosis
High fructose causes cardiac hypertrophy via mitochondrial signaling pathway.
Cystic Fibrosis
High incidence of the CFTR mutations 3272-26A-->G and L927P in Belgian cystic fibrosis patients, and identification of three new CFTR mutations (186-2A-->G, E588V, and 1671insTATCA).
Cystic Fibrosis
High level of CFTR expression is associated with tumor aggression and knockdown of CFTR suppresses proliferation of ovarian cancer in vitro and in vivo.
Cystic Fibrosis
High-affinity activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening.
Cystic Fibrosis
High-density multiplex detection of nucleic acid sequences: oligonucleotide ligation assay and sequence-coded separation.
Cystic Fibrosis
High-efficiency transfer of cystic fibrosis transmembrane conductance regulator cDNA into cystic fibrosis airway cells in culture using lactosylated polylysine as a vector.
Cystic Fibrosis
High-Efficiency, Selection-free Gene Repair in Airway Stem Cells from Cystic Fibrosis Patients Rescues CFTR Function in Differentiated Epithelia.
Cystic Fibrosis
High-expressing cystic fibrosis transmembrane conductance regulator interacts with histone deacetylase 2 to promote the development of Ph+ leukemia through the HDAC2-mediated PTEN pathway.
Cystic Fibrosis
High-fat diet modifies the PPAR-? pathway leading to disruption of microbial and physiological ecosystem in murine small intestine.
Cystic Fibrosis
High-Potency Phenylquinoxalinone Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activators.
Cystic Fibrosis
High-resolution imaging of the actin cytoskeleton and epithelial sodium channel, CFTR, and aquaporin-9 localization in the vas deferens.
Cystic Fibrosis
High-Throughput Screening for Modulators of CFTR Activity Based on Genetically Engineered Cystic Fibrosis Disease-Specific iPSCs.
Cystic Fibrosis
High-Throughput Screening for Readthrough Modulators of CFTR PTC Mutations.
Cystic Fibrosis
High-throughput screening identifies FAU protein as a regulator of mutant cystic fibrosis transmembrane conductance regulator channel.
Cystic Fibrosis
Higher proportion of intact exon 9 CFTR mRNA in nasal epithelium compared with vas deferens.
Cystic Fibrosis
Histamine 1 receptor-G??-cAMP/PKA-CFTR pathway mediates the histamine-induced resetting of the suprachiasmatic circadian clock.
Cystic Fibrosis
Histological and genetic analysis and risk assessment for chromosomal aberration after ICSI for patients presenting with CBAVD.
Cystic Fibrosis
History of cryptorchidism and ejaculate volume as simple predictors for the presence of testicular sperm.
Cystic Fibrosis
HIV Infects Bronchial Epithelium and Suppresses Components of the Mucociliary Clearance Apparatus.
Cystic Fibrosis
HLA class II polymorphism in cystic fibrosis. A possible modifier of pulmonary phenotype.
Cystic Fibrosis
Homogeneity of Aerosol Deposition and Mucociliary Clearance are Improved Following Ivacaftor Treatment in Cystic Fibrosis.
Cystic Fibrosis
Homology Requirements for Efficient, Footprintless Gene Editing at the CFTR Locus in Human iPSCs with Helper-dependent Adenoviral Vectors.
Cystic Fibrosis
Homozygosity for a novel splice site mutation (2790-2 A--->G) preceding exon 15 of the CFTR gene in a cystic fibrosis patient of North-East Italian descent.
Cystic Fibrosis
Homozygosity for L997F in a child with normal clinical and chloride secretory phenotype provides evidence that this cystic fibrosis transmembrane conductance regulator mutation does not cause cystic fibrosis.
Cystic Fibrosis
Homozygous (TG)11 allele in intron 8 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene has a protective role against bicarbonate decrease in pure pancreatic juice among Japanese male alcoholics.
Cystic Fibrosis
Homozygous Deletion of the CFTR Gene Caused by Interstitial Maternal Isodisomy in a Peruvian Child with Cystic Fibrosis.
Cystic Fibrosis
Hormonal control of the renal immune response and antibacterial host defense by arginine vasopressin.
Cystic Fibrosis
Hormonal regulation of cardiac cystic fibrosis transmembrane conductance regulator chloride channels.
Cystic Fibrosis
Host mucin glycosylation plays a role in bacterial adhesion in lungs of individuals with cystic fibrosis.
Cystic Fibrosis
Host resistance to lung infection mediated by major vault protein in epithelial cells.
Cystic Fibrosis
House dust mite extract activates apical Cl(-) channels through protease-activated receptor 2 in human airway epithelia.
Cystic Fibrosis
How Clinically Efficient Is Lumacaftor/Ivacaftor for Cystic Fibrosis Patients? An Updated Literature Review.
Cystic Fibrosis
How do cystic fibrosis transmembrane conductance regulator mutations produce lung disease?
Cystic Fibrosis
How mutant CFTR may contribute to Pseudomonas aeruginosa infection in cystic fibrosis.
Cystic Fibrosis
How Phosphorylation and ATPase Activity Regulate Anion Flux though the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Cystic Fibrosis
How phosphorylation and ATPase activity regulate anion flux through the cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
How the airway smooth muscle in cystic fibrosis reacts in proinflammatory conditions: implications for airway hyper-responsiveness and asthma in cystic fibrosis.
Cystic Fibrosis
Hsp104 facilitates the endoplasmic-reticulum-associated degradation of disease-associated and aggregation-prone substrates.
Cystic Fibrosis
Hsp70 molecular chaperone facilitates endoplasmic reticulum-associated protein degradation of cystic fibrosis transmembrane conductance regulator in yeast.
Cystic Fibrosis
Human alveolar type II cells secrete and absorb liquid in response to local nucleotide signaling.
Cystic Fibrosis
Human amnion epithelial cells induced to express functional cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Human CFTR gene sequences in regions flanking exon 10: a simple repeat sequence polymorphism in intron 9.
Cystic Fibrosis
Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance.
Cystic Fibrosis
Human cystic fibrosis monocyte derived macrophages display no defect in acidification of phagolysosomes when measured by optical nanosensors.
Cystic Fibrosis
Human cystic fibrosis transmembrane conductance regulator directed to respiratory epithelial cells of transgenic mice.
Cystic Fibrosis
Human Enteroids as a Model of Upper Small Intestinal Ion Transport Physiology and Pathophysiology.
Cystic Fibrosis
Human epithelial cystic fibrosis transmembrane conductance regulator without exon 5 maintains partial chloride channel function in intracellular membranes.
Cystic Fibrosis
Human extrahepatic and intrahepatic cholangiocyte organoids show region-specific differentiation potential and model cystic fibrosis-related bile duct disease.
Cystic Fibrosis
Human fetal trachea-SCID mouse xenografts: efficacy of vesicular stomatitis virus-G pseudotyped lentiviral-mediated gene transfer.
Cystic Fibrosis
Human Genetics Society of Australasia position statement: population-based carrier screening for cystic fibrosis.
Cystic Fibrosis
Human heat shock protein 105/110 kDa (Hsp105/110) regulates biogenesis and quality control of misfolded cystic fibrosis transmembrane conductance regulator at multiple levels.
Cystic Fibrosis
Human lymphocytes transcribe the cystic fibrosis transmembrane conductance regulator gene and exhibit CF-defective cAMP-regulated chloride current.
Cystic Fibrosis
Human Nasal Epithelial Organoids for Therapeutic Development in Cystic Fibrosis.
Cystic Fibrosis
Human pluripotent stem cell-derived acinar/ductal organoids generate human pancreas upon orthotopic transplantation and allow disease modelling.
Cystic Fibrosis
Human Sperm Capacitation Involves the Regulation of the Tyr-Phosphorylation Level of the Anion Exchanger 1 (AE1).
Cystic Fibrosis
Human, Drosophila, and C.elegans TDP43: nucleic acid binding properties and splicing regulatory function.
Cystic Fibrosis
Human-mouse cystic fibrosis transmembrane conductance regulator (CFTR) chimeras identify regions that partially rescue CFTR-?F508 processing and alter its gating defect.
Cystic Fibrosis
Human-specific cystic fibrosis transmembrane conductance regulator antibodies detect in vivo gene transfer to ovine airways.
Cystic Fibrosis
Humoral and cellular immune responses of nonhuman primates to long-term repeated lung exposure to Ad2/CFTR-2.
Cystic Fibrosis
Huqi San-Evoked Rat Colonic Anion Secretion through Increasing CFTR Expression.
Cystic Fibrosis
Hybrid Nonviral/Viral Vector Systems for Improved piggyBac DNA Transposon In Vivo Delivery.
Cystic Fibrosis
Hybridization of glass-tethered oligonucleotide probes to target strands preannealed with labeled auxiliary oligonucleotides.
Cystic Fibrosis
Hydrogen peroxide stimulates chloride secretion in primary inner medullary collecting duct cells via mPGES-1-derived PGE2.
Cystic Fibrosis
Hydrogen Sulfide Facilitates Vaginal Lubrication by Activation of Epithelial ATP-Sensitive K(+) Channels and Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
Hydrogen sulfide stimulates CFTR in Xenopus oocytes by activation of the cAMP/PKA signalling axis.
Cystic Fibrosis
Hydrogen sulfide: A novel signaling molecule in the central nervous system.
Cystic Fibrosis
Hydrolytic and nonhydrolytic interactions in the ATP regulation of CFTR Cl- conductance.
Cystic Fibrosis
Hyperabsorption of Na+ and raised Ca(2+)-mediated Cl- secretion in nasal epithelia of CF mice.
Cystic Fibrosis
Hyperacidification of cellubrevin endocytic compartments and defective endosomal recycling in cystic fibrosis respiratory epithelial cells.
Cystic Fibrosis
Hypercapnia modulates cAMP signalling and cystic fibrosis transmembrane conductance regulator-dependent anion and fluid secretion in airway epithelia.
Cystic Fibrosis
Hyperexpression of recombinant CFTR in heterologous cells alters its physiological properties.
Cystic Fibrosis
Hypertonic saline increases lung epithelial lining fluid glutathione and thiocyanate: two protective CFTR-dependent thiols against oxidative injury.
Cystic Fibrosis
Hypertonic saline releases the attached small intestinal cystic fibrosis mucus.
Cystic Fibrosis
Hypertonic saline solutions do not influence the solubility of sputum from secretor and non-secretor cystic fibrosis patients.
Cystic Fibrosis
Hypochloremia and hyponatremia as the initial presentation of cystic fibrosis in three adults.
Cystic Fibrosis
Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands.
Cystic Fibrosis
Hypothesis: vitamin E complements polyunsaturated fatty acids in essential fatty acid deficiency in cystic fibrosis.
Cystic Fibrosis
Hypothetical framework for enhanced renal tubular secretion of drugs in cystic fibrosis.
Cystic Fibrosis
Hypoxia increases corneal cell expression of CFTR leading to increased Pseudomonas aeruginosa binding, internalization, and initiation of inflammation.
Cystic Fibrosis
Hypoxia inducible factor-1 (HIF-1)-mediated repression of cystic fibrosis transmembrane conductance regulator (CFTR) in the intestinal epithelium.
Cystic Fibrosis
Ibuprofen rescues mutant cystic fibrosis transmembrane conductance regulator trafficking.
Cystic Fibrosis
ICAM-1 expression on conjunctival epithelial cells in patients with cystic fibrosis.
Cystic Fibrosis
Identical intragenic microsatellite haplotype found in cystic fibrosis chromosomes bearing mutation G551D in Irish, English, Scottish, Breton and Czech patients.
Cystic Fibrosis
Identification and characterization of CFTR gene mutations in Indian CF patients.
Cystic Fibrosis
Identification and developmental expression of the Xenopus laevis cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Identification and localization of a dogfish homolog of human cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Identification and membrane localization of electrogenic sodium bicarbonate cotransporters in Calu-3 cells.
Cystic Fibrosis
Identification and partial characterization of a domain in CFTR that may bind cyclic nucleotides directly.
Cystic Fibrosis
Identification and regulation of the cystic fibrosis transmembrane conductance regulator-generated chloride channel.
Cystic Fibrosis
Identification of a new cystic fibrosis transmembrane regulator mutation in a severely affected patient.
Cystic Fibrosis
Identification of a Novel 5' Alternative CFTR mRNA Isoform in a Patient with Nasal Polyposis and CFTR Mutations.
Cystic Fibrosis
Identification of a Novel Frame-Shift Mutation in PRSS1 Gene in Han Patients with Autoimmune Pancreatitis.
Cystic Fibrosis
Identification of a novel functional miR-143-5p recognition element in the Cystic Fibrosis Transmembrane Conductance Regulator 3'UTR.
Cystic Fibrosis
Identification of a novel large deletion and other copy number variations in the CFTR gene in patients with Cystic Fibrosis from a multiethnic population.
Cystic Fibrosis
Identification of a novel missense mutation (G314E) in exon 7 of the cystic fibrosis transmembrane conductance regulator gene identified in a CF patient with pancreatic sufficiency.
Cystic Fibrosis
Identification of a novel mutation of CFTR gene in a Korean patient with cystic fibrosis.
Cystic Fibrosis
Identification of a novel nonsense mutation (L88X) in exon 3 of the cystic fibrosis transmembrane conductance regulator gene in a native Korean cystic fibrosis chromosome.
Cystic Fibrosis
Identification of a novel SERPINA-1 mutation causing alpha-1 antitrypsin deficiency in a patient with severe bronchiectasis and pulmonary embolism.
Cystic Fibrosis
Identification of a novel water soluble activator of wild-type and F508del CFTR: GPact-11a.
Cystic Fibrosis
Identification of a protein that confers calcitonin gene-related peptide responsiveness to oocytes by using a cystic fibrosis transmembrane conductance regulator assay.
Cystic Fibrosis
Identification of a second blocker binding site at the cytoplasmic mouth of the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Cystic Fibrosis
Identification of an 11T allele in the polypyrimidine tract of intron 8 of the CFTR gene.
Cystic Fibrosis
Identification of an Anti-Inflammation Protein, Annexin A1, in Tendon Derived Stem Cells (TDSCs) of Cystic Fibrosis Mice: A Comparative Proteomic Analysis.
Cystic Fibrosis
Identification of an ion channel-forming motif in the primary structure of CFTR, the cystic fibrosis chloride channel.
Cystic Fibrosis
Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Identification of CFTR, PRSS1, and SPINK1 mutations in 381 patients with pancreatitis.
Cystic Fibrosis
Identification of Compounds That Promote Readthrough of Premature Termination Codons in the CFTR.
Cystic Fibrosis
Identification of cystic fibrosis transmembrane conductance regulator channel-lining residues in and flanking the M6 membrane-spanning segment.
Cystic Fibrosis
Identification of cystic fibrosis transmembrane conductance regulator in renal endosomes.
Cystic Fibrosis
Identification of eight mutations and three sequence variations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
Identification of four new mutations in the cystic fibrosis transmembrane conductance regulator gene: I148T, L1077P, Y1092X, 2183AA-->G.
Cystic Fibrosis
Identification of four novel mutations in the cystic fibrosis transmembrane conductance regulator gene: E664X, 2113delA, 306delTAGA, and delta M1140.
Cystic Fibrosis
Identification of Gasz, an evolutionarily conserved gene expressed exclusively in germ cells and encoding a protein with four ankyrin repeats, a sterile-alpha motif, and a basic leucine zipper.
Cystic Fibrosis
Identification of genes and pathways in esophageal adenocarcinoma using bioinformatics analysis.
Cystic Fibrosis
Identification of GLPG/ABBV-2737, a Novel Class of Corrector, Which Exerts Functional Synergy With Other CFTR Modulators.
Cystic Fibrosis
Identification of key pathways and genes in Barrett's esophagus using integrated bioinformatics methods.
Cystic Fibrosis
Identification of molecular determinants that modulate trafficking of DeltaF508 CFTR, the mutant ABC transporter associated with cystic fibrosis.
Cystic Fibrosis
Identification of mutations in exons 1 through 8 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
Identification of natural coumarin compounds that rescue defective DeltaF508-CFTR chloride channel gating.
Cystic Fibrosis
Identification of new small molecule inhibitors of cystic fibrosis transmembrane conductance regulator protein: in vitro and in vivo studies.
Cystic Fibrosis
Identification of novel and rare mutations in California Hispanic and African American cystic fibrosis patients.
Cystic Fibrosis
Identification of novel mutations in Arabs with cystic fibrosis and their impact on the cystic fibrosis transmembrane regulator mutation detection rate in Arab populations.
Cystic Fibrosis
Identification of patients with genetic risk factors of pancreatitis: impact on treatment and cancer prevention.
Cystic Fibrosis
Identification of positive charges situated at the outer mouth of the CFTR chloride channel pore.
Cystic Fibrosis
Identification of Potential Leukocyte Biomarkers Related to Drug Recovery of CFTR: Clinical Applications in Cystic Fibrosis.
Cystic Fibrosis
Identification of protein kinase A phosphorylation sites on NBD1 and R domains of CFTR using electrospray mass spectrometry with selective phosphate ion monitoring.
Cystic Fibrosis
Identification of resveratrol oligomers as inhibitors of cystic fibrosis transmembrane conductance regulator by high-throughput screening of natural products from chinese medicinal plants.
Cystic Fibrosis
Identification of revertants for the cystic fibrosis delta F508 mutation using STE6-CFTR chimeras in yeast.
Cystic Fibrosis
Identification of RL-TGR, a coreceptor involved in aversive chemical signaling.
Cystic Fibrosis
Identification of six mutations (R31L, 441delA, 681delC, 1461ins4, W1089R, E1104X) in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
Identification of specific sites in human P-glycoprotein phosphorylated by protein kinase C.
Cystic Fibrosis
Identification of synergistic combinations of F508del cystic fibrosis transmembrane conductance regulator (CFTR) modulators.
Cystic Fibrosis
Identification of the 5T-12TG allele of the cystic fibrosis transmembrane conductance regulator gene in hypertrypsinaemic newborns.
Cystic Fibrosis
Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequences.
Cystic Fibrosis
Identification of the cofilin-binding sites in the large cytoplasmic domain of Na,K-ATPase.
Cystic Fibrosis
Identification of the commonest cystic fibrosis transmembrane regulator gene DeltaF508 mutation: evaluation of PCR--single-strand conformational polymorphism and polyacrylamide gel electrophoresis.
Cystic Fibrosis
Identification of the cystic fibrosis transmembrane conductance regulator domains that are important for interactions with ROMK2.
Cystic Fibrosis
Identification of the five most common cystic fibrosis mutations in single cells using a rapid and specific differential amplification system.
Cystic Fibrosis
Identification of the human cortactin-binding protein-2 gene from the autism candidate region at 7q31.
Cystic Fibrosis
Identification of the linkage of mutations causing cystic fibrosis to different alleles of a tetranucleotide repeat in intron 6a of the CFTR gene.
Cystic Fibrosis
Identification of the M1101K mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and complete detection of cystic fibrosis mutations in the Hutterite population.
Cystic Fibrosis
Identification of the major phosphorylation domain of murine mdr1b P-glycoprotein. Analysis of the protein kinase A and protein kinase C phosphorylation sites.
Cystic Fibrosis
Identification of the Novel TMEM16A Inhibitor Dehydroandrographolide and Its Anticancer Activity on SW620 Cells.
Cystic Fibrosis
Identification of three novel mutations (457 TAT-->G, D192G, Q685X) in the Slovenian CF patients.
Cystic Fibrosis
Identification of three novel mutations in the CFTR gene using temperature-optimized non-radioactive conditions for SSCP analysis.
Cystic Fibrosis
Identification of three novel mutations in the cystic fibrosis transmembrane conductance regulator gene in Argentinian CF patients.
Cystic Fibrosis
Identification of transport abnormalities in duodenal mucosa and duodenal enterocytes from patients with cystic fibrosis.
Cystic Fibrosis
Idiopathic CD4+ T lymphocytopenia presenting as mycetoma in a patient with a mutation in the cystic fibrosis transmembrane regulator gene.
Cystic Fibrosis
IGF binding protein-3 treatment alters intestinal cell proliferation but not body weight of adult cystic fibrosis transmembrane conductance regulator deficient mice.
Cystic Fibrosis
IL-17 primes airway epithelial cells lacking functional Cystic Fibrosis Transmembrane conductance Regulator (CFTR) to increase NOD1 responses.
Cystic Fibrosis
IL-4 is a potent modulator of ion transport in the human bronchial epithelium in vitro.
Cystic Fibrosis
IL-8 inhibits cAMP-stimulated alveolar epithelial fluid transport via a GRK2/PI3K-dependent mechanism.
Cystic Fibrosis
Image-based ?-adrenergic sweat rate assay captures minimal cystic fibrosis transmembrane conductance regulator function.
Cystic Fibrosis
Immortalization of subpopulations of respiratory epithelial cells from transgenic mice bearing SV40 large T antigen.
Cystic Fibrosis
Immortalized bovine pancreatic duct cells become tumorigenic after transfection with mutant k-ras.
Cystic Fibrosis
Immortalized epithelial cells from human autosomal dominant polycystic kidney cysts.
Cystic Fibrosis
Immortalized intrahepatic mouse biliary epithelial cells: immunologic characterization and immunogenicity.
Cystic Fibrosis
Immune mediators regulate CFTR expression through a bifunctional airway-selective enhancer.
Cystic Fibrosis
Immune response of polarized cystic fibrosis airway epithelial cells infected with Influenza A virus.
Cystic Fibrosis
Immuno and functional characterization of CFTR in submandibular and pancreatic acinar and duct cells.
Cystic Fibrosis
Immunocytochemical analysis reveals differences between the subcellular localization of normal and delta Phe508 recombinant cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Immunohistochemical analysis of the distribution of molecules involved in ionic and pH regulation in the lancelet Branchiostoma floridae (Hubbs, 1922).
Cystic Fibrosis
Immunohistochemical and electron microscopic characterization of brush cells of the rat cecum.
Cystic Fibrosis
Immunohistochemical Detection of Markers for Translational Studies of Lung Disease in Pigs and Humans.
Cystic Fibrosis
Immunohistochemical localization of cystic fibrosis transmembrane conductance regulator in human fetal airway and digestive mucosa.
Cystic Fibrosis
Immunohistochemical localization of cystic fibrosis transmembrane regulator and clara cell secretory protein in taste receptor cells of rat circumvallate papillae.
Cystic Fibrosis
Immunohistochemical localization of guanylin in the rat small intestine and colon.
Cystic Fibrosis
Immunohystochemical analysis of CFTR in normal and disrupted spermatogenesis.
Cystic Fibrosis
Immunolocalization and regulation of cystic fibrosis transmembrane conductance regulator in the adult rat epididymis.
Cystic Fibrosis
Immunolocalization of chloride transporters to gill epithelia of euryhaline teleosts with opposite salinity-induced Na(+)/K (+)-ATPase responses.
Cystic Fibrosis
Immunolocalization of ion transport proteins in human autosomal dominant polycystic kidney epithelial cells.
Cystic Fibrosis
Immunolocalization of ion-transport proteins to branchial epithelium mitochondria-rich cells in the mudskipper (Periophthalmodon schlosseri).
Cystic Fibrosis
Immunologic responses to gene transfer into mice via the polymeric immunoglobulin receptor.
Cystic Fibrosis
Immunopathology and immunogenetics of allergic bronchopulmonary aspergillosis.
Cystic Fibrosis
Impact of a cystic fibrosis transmembrane conductance regulator (CFTR) modulator on high-dose ibuprofen therapy in pediatric cystic fibrosis patients.
Cystic Fibrosis
Impact of CFTR DeltaF508 mutation on prostaglandin E2 production and type IIA phospholipase A2 expression by pulmonary epithelial cells.
Cystic Fibrosis
Impact of CFTR modulation with Ivacaftor on Gut Microbiota and Intestinal Inflammation.
Cystic Fibrosis
Impact of CFTR-modulating drugs on GH-IGF-1 axis impairment in adult patients with cystic fibrosis.
Cystic Fibrosis
Impact of cystic fibrosis transmembrane conductance regulator gene mutation on the occurrence of chronic pancreatitis in Japanese patients.
Cystic Fibrosis
Impact of Cystic Fibrosis Transmembrane Regulator (CFTR) gene mutations on male infertility.
Cystic Fibrosis
Impact of heterogeneity within cultured cells on bacterial invasion: analysis of Pseudomonas aeruginosa and Salmonella enterica serovar typhi entry into MDCK cells by using a green fluorescent protein-labelled cystic fibrosis transmembrane conductance regulator receptor.
Cystic Fibrosis
Impact of high- versus low-risk genotype on sinonasal radiographic disease in cystic fibrosis.
Cystic Fibrosis
Impact of hypoxia and AMPK on CFTR-mediated bicarbonate secretion in human cholangiocyte organoids.
Cystic Fibrosis
Impact of mannose binding lectin (MBL) insufficiency on the course of cystic fibrosis: a review and meta-analysis.
Cystic Fibrosis
Impact of pharmacy services on cystic fibrosis transmembrane conductance regulator modulator prescribing at a pediatric cystic fibrosis center.
Cystic Fibrosis
Impact of Physical Activity on Cognitive Functions: A New Field for Research and Management of Cystic Fibrosis.
Cystic Fibrosis
Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth.
Cystic Fibrosis
Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure.
Cystic Fibrosis
Impact of the F508del mutation on ovine CFTR, a Cl(-) channel with enhanced conductance and ATP-dependent gating.
Cystic Fibrosis
Impaired cardiac and peripheral hemodynamic responses to inhaled ??-agonist in cystic fibrosis.
Cystic Fibrosis
Impaired cell volume regulation in intestinal crypt epithelia of cystic fibrosis mice.
Cystic Fibrosis
Impaired CFTR-Dependent Amplification of FSH-Stimulated Estrogen Production in Cystic Fibrosis and PCOS.
Cystic Fibrosis
Impaired regulatory volume decrease in freshly isolated cholangiocytes from cystic fibrosis mice: implications for cystic fibrosis transmembrane conductance regulator effect on potassium conductance.
Cystic Fibrosis
Impaired transporter associated with antigen processing (TAP) function attributable to a single amino acid alteration in the peptide TAP subunit TAP1.
Cystic Fibrosis
Implementation of newborn screening for cystic fibrosis in Norway. Results from the first three years.
Cystic Fibrosis
Implication of the cystic fibrosis transmembrane conductance regulator gene in infertile family members of Indian CF patients.
Cystic Fibrosis
Importance of basolateral K+ conductance in maintaining Cl- secretion in murine nasal and colonic epithelia.
Cystic Fibrosis
Importance of SLC26 Transmembrane Anion Exchangers in Sperm Post-testicular Maturation and Fertilization Potential.
Cystic Fibrosis
Improved correction of F508del-CFTR biogenesis with a folding facilitator and an inhibitor of protein ubiquitination.
Cystic Fibrosis
Improved detection of CFTR mutations in Southern California Hispanic CF patients.
Cystic Fibrosis
Improved detection of cystic fibrosis mutations in infertility patients with DNA sequence analysis.
Cystic Fibrosis
Improved fluorescence assays to measure the defects associated with F508del-CFTR allow identification of new active compounds.
Cystic Fibrosis
Improved oxygenation promotes CFTR maturation and trafficking in MDCK monolayers.
Cystic Fibrosis
Improvement in exercise duration, lung function and well-being in G551D-cystic fibrosis patients: a double-blind, placebo-controlled, randomized, cross-over study with ivacaftor treatment.
Cystic Fibrosis
Improving complex medical care while awaiting next-generation CFTR potentiators and correctors: The current pipeline of therapeutics.
Cystic Fibrosis
Improving newborn screening for cystic fibrosis using next-generation sequencing technology: a technical feasibility study.
Cystic Fibrosis
Improving outcomes of infections in cystic fibrosis in the era of CFTR modulator therapy.
Cystic Fibrosis
In cellulo analyses of the p.Val322Ala mutation on the CFTR protein conformation and activity.
Cystic Fibrosis
In NF1, CFTR, PER3, CARS and SYT7, alternatively included exons show higher conservation of surrounding intron sequences than constitutive exons.
Cystic Fibrosis
In silico drug repositioning on F508del-CFTR: A proof-of-concept study on the AIFA library.
Cystic Fibrosis
In silico prediction and validation of potential therapeutic genes in pancreatic ?-cells associated with type 2 diabetes.
Cystic Fibrosis
In utero and postnatal VX-770 administration rescues multiorgan disease in a ferret model of cystic fibrosis.
Cystic Fibrosis
In vitro 3D culture lung model from expanded primary cystic fibrosis human airway cells.
Cystic Fibrosis
In Vitro Analysis of PDZ-dependent CFTR Macromolecular Signaling Complexes.
Cystic Fibrosis
In vitro and in vivo functional characterization of gutless recombinant SV40-derived CFTR vectors.
Cystic Fibrosis
In vitro assessment of variables affecting the efficiency and efficacy of adenovirus-mediated gene transfer to cystic fibrosis airway epithelia.
Cystic Fibrosis
In vitro evaluation of drug delivery behavior for inhalable amorphous nanoparticle formulations in a human lung epithelial cell model.
Cystic Fibrosis
In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.
Cystic Fibrosis
In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study.
Cystic Fibrosis
In Vitro Recovery of ATP-Sensitive Potassium Channels in {beta}-Cells From Patients With Congenital Hyperinsulinism of Infancy.
Cystic Fibrosis
In vitro validation of duct differentiation in developing embryonic mouse pancreas.
Cystic Fibrosis
In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium.
Cystic Fibrosis
In vivo and in vitro correction of the mdx dystrophin gene nonsense mutation by short-fragment homologous replacement.
Cystic Fibrosis
In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies.
Cystic Fibrosis
In vivo cell-specific expression of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
In vivo evaluation of the safety of adenovirus-mediated transfer of the human cystic fibrosis transmembrane conductance regulator cDNA to the lung.
Cystic Fibrosis
In vivo investigation of acidified pepsin exposure to porcine vocal fold epithelia.
Cystic Fibrosis
In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis.
Cystic Fibrosis
In vivo pharmacology and antidiarrheal efficacy of a thiazolidinone CFTR inhibitor in rodents.
Cystic Fibrosis
In vivo transfer of the human cystic fibrosis transmembrane conductance regulator gene to the airway epithelium.
Cystic Fibrosis
In-vitro evaluation of a ciprofloxacin- and ivacaftor-coated sinus stent against Pseudomonas aeruginosa biofilms.
Cystic Fibrosis
In-vivo crystals reveal critical features of the interaction between CFTR and the PDZ2 domain of Na+/H+ exchange cofactor NHERF1.
Cystic Fibrosis
In-vivo functional study on the involvement of CFTR, SLC26A6, NHE-1 and CA isoenzymes II and XII in uterine fluid pH, volume and electrolyte regulation in rats under different sex-steroid influence.
Cystic Fibrosis
Incidence, etiology and prognosis of first-time acute pancreatitis in young patients: a population-based cohort study.
Cystic Fibrosis
Incidence, Prevalence, Etiology, and Prognosis of First-Time Chronic Pancreatitis in Young Patients: A Nationwide Cohort Study.
Cystic Fibrosis
Incomplete reprogramming after fusion of human multipotent stromal cells and bronchial epithelial cells.
Cystic Fibrosis
Incomplete rescue of cystic fibrosis transmembrane conductance regulator deficient mice by the human CFTR cDNA.
Cystic Fibrosis
Increase in intracellular Cl- concentration by cAMP- and Ca2+-dependent stimulation of M1 collecting duct cells.
Cystic Fibrosis
Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice.
Cystic Fibrosis
Increased apical Na+ permeability in cystic fibrosis is supported by a quantitative model of epithelial ion transport.
Cystic Fibrosis
Increased arylsulfatase B activity in cystic fibrosis cells following correction of CFTR.
Cystic Fibrosis
Increased chloride efflux in colchicine-resistant airway epithelial cell lines.
Cystic Fibrosis
Increased contact time improves adenovirus-mediated CFTR gene transfer to nasal epithelium of CF mice.
Cystic Fibrosis
Increased cystic fibrosis transmembrane conductance regulator (CFTR) expression in the human hydrosalpinx.
Cystic Fibrosis
Increased diffusional mobility of CFTR at the plasma membrane after deletion of its C-terminal PDZ binding motif.
Cystic Fibrosis
Increased expression of cystic fibrosis transmembrane conductance regulator in rat liver after common bile duct ligation.
Cystic Fibrosis
Increased expression of sulfated gp300 and acinar tissue pathology in pancreas of CFTR(-/-) mice.
Cystic Fibrosis
Increased expression of the secretory Na+-K+-2Cl- cotransporter with differentiation of a human intestinal cell line.
Cystic Fibrosis
Increased extracellular vesicles mediate inflammatory signalling in cystic fibrosis.
Cystic Fibrosis
Increased Fecal Calprotectin Is Associated with Worse Gastrointestinal Symptoms and Quality of Life Scores in Children with Cystic Fibrosis.
Cystic Fibrosis
Increased Folding and Channel Activity of a Rare Cystic Fibrosis Mutant with CFTR Modulators.
Cystic Fibrosis
Increased frequency of CFTR gene mutations identified in Indian infertile men with non-CBAVD obstructive azoospermia and spermatogenic failure.
Cystic Fibrosis
Increased frequency of cystic fibrosis deltaF508 mutation in bronchiectasis associated with rheumatoid arthritis.
Cystic Fibrosis
Increased frequency of cystic fibrosis transmembrane conductance regulator gene mutations in infertile males.
Cystic Fibrosis
Increased functional cell surface expression of CFTR and DeltaF508-CFTR by the anthracycline doxorubicin.
Cystic Fibrosis
Increased incidence of cystic fibrosis gene mutations in adults with disseminated bronchiectasis.
Cystic Fibrosis
Increased intracellular Cl- concentration mediates Trichomonas vaginalis-induced inflammation in the human vaginal epithelium.
Cystic Fibrosis
Increased NaCl-induced interleukin-8 production by human bronchial epithelial cells is enhanced by the DeltaF508/W1282X mutation of the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Increased NF-?B Activity and Decreased Wnt/?-Catenin Signaling Mediate Reduced Osteoblast Differentiation and Function in ?F508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mice.
Cystic Fibrosis
Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis.
Cystic Fibrosis
Increased prevalence of mutations in the cystic fibrosis transmembrane conductance regulator in children with chronic rhinosinusitis.
Cystic Fibrosis
Increases in apoptosis, caspase activity and expression of p53 and bax, and the transition between two types of mitochondrion-rich cells, in the gills of the climbing perch, Anabas testudineus, during a progressive acclimation from freshwater to seawater.
Cystic Fibrosis
Increases in cytosolic Ca2+ induce dynamin- and calcineurin-dependent internalisation of CFTR.
Cystic Fibrosis
Increasing the Endoplasmic Reticulum Pool of the F508del Allele of the Cystic Fibrosis Transmembrane Conductance Regulator Leads to Greater Folding Correction by Small Molecule Therapeutics.
Cystic Fibrosis
Indazole inhibition of cystic fibrosis transmembrane conductance regulator Cl(-) channels in rat epididymal epithelial cells.
Cystic Fibrosis
Individualized medicine using intestinal responses to CFTR potentiators and correctors.
Cystic Fibrosis
Induction of a cAMP-stimulated chloride secretion in regenerating poorly differentiated airway epithelial cells by adenovirus-mediated CFTR gene transfer.
Cystic Fibrosis
Induction of CFTR gene expression by 1,25(OH)2 vitamin D3, 25OH vitamin D3, and vitamin D3 in cultured human airway epithelial cells and in mouse airways.
Cystic Fibrosis
Induction of expression of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Induction of multidrug resistance downregulates the expression of CFTR in colon epithelial cells.
Cystic Fibrosis
Inefficient gene transfer by adenovirus vector to cystic fibrosis airway epithelia of mice and humans.
Cystic Fibrosis
Infection by Toxoplasma gondii, a severe parasite in neonates and AIDS patients, causes impaired anion secretion in airway epithelia.
Cystic Fibrosis
Infection of Polarized Airway Epithelial Cells by Normal and Small-Colony Variant Strains of Staphylococcus aureus Is Increased in Cells with Abnormal Cystic Fibrosis Transmembrane Conductance Regulator Function and Is Influenced by NF-{kappa}B.
Cystic Fibrosis
Infertility in females with cystic fibrosis is multifactorial: evidence from mouse models.
Cystic Fibrosis
Inflammation in cystic fibrosis airways: relationship to increased bacterial adherence.
Cystic Fibrosis
Inflammatory cytokines TNF-? and IL-17 enhance the efficacy of cystic fibrosis transmembrane conductance regulator modulators.
Cystic Fibrosis
Inflammatory epithelial cytokines after in vitro respiratory syncytial viral infection are associated with reduced lung function.
Cystic Fibrosis
Influence of cystic fibrosis transmembrane conductance regulator on gene expression in response to Pseudomonas aeruginosa infection of human bronchial epithelial cells.
Cystic Fibrosis
Influence of salinity on the localization and expression of the CFTR chloride channel in the ionocytes of Dicentrarchus labrax during ontogeny.
Cystic Fibrosis
Influence of salinity on the localization of Na+/K+-ATPase, Na+/K+/2Cl- cotransporter (NKCC) and CFTR anion channel in chloride cells of the Hawaiian goby (Stenogobius hawaiiensis).
Cystic Fibrosis
Influence of the cystic fibrosis transmembrane conductance regulator on expression of lipid metabolism-related genes in dendritic cells.
Cystic Fibrosis
Influence of the duplication of CFTR exon 9 and its flanking sequences on diagnosis of cystic fibrosis mutations.
Cystic Fibrosis
Influenza Matrix Protein 2 Alters CFTR Expression and Function through its Ion Channel Activity.
Cystic Fibrosis
Influenza virus infection alters ion channel function of Airway and alveolar cells: Mechanisms and physiological sequelae.
Cystic Fibrosis
Influenza virus M2 targets cystic fibrosis transmembrane conductance regulator for lysosomal degradation during viral infection.
Cystic Fibrosis
Influenza-mediated reduction of lung epithelial ion channel activity leads to dysregulated pulmonary fluid homeostasis.
Cystic Fibrosis
Infrared biomarkers of impaired cystic fibrosis transmembrane regulator protein biogenesis.
Cystic Fibrosis
Inhaled interventions in cystic fibrosis: mucoactive and antibiotic therapies.
Cystic Fibrosis
Inhaled phosphodiesterase type 5 inhibitors for cystic fibrosis: a new therapy for systemic disease?
Cystic Fibrosis
Inhibiting endoplasmic reticulum (ER)-associated degradation of misfolded Yor1p does not permit ER export despite the presence of a diacidic sorting signal.
Cystic Fibrosis
Inhibition by TNF-alpha and IL-4 of cationic lipid mediated gene transfer in cystic fibrosis tracheal gland cells.
Cystic Fibrosis
Inhibition of Alk signaling promotes the induction of human salivary-gland-derived organoids.
Cystic Fibrosis
Inhibition of amiloride-sensitive Na(+) absorption by activation of CFTR in mouse endometrial epithelium.
Cystic Fibrosis
Inhibition of ATP-sensitive K+ channels by substituted benzo[c]quinolizinium CFTR activators.
Cystic Fibrosis
Inhibition of ATPase, GTPase and adenylate kinase activities of the second nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator by genistein.
Cystic Fibrosis
Inhibition of calpain blocks the phagosomal escape of Listeria monocytogenes.
Cystic Fibrosis
Inhibition of cAMP- and Ca-dependent Cl- secretion by phorbol esters: inhibition of basolateral K+ channels.
Cystic Fibrosis
Inhibition of CFTR-mediated intestinal chloride secretion as potential therapy for bile acid diarrhea.
Cystic Fibrosis
Inhibition of CFTR-mediated intestinal chloride secretion by a fungus-derived arthropsolide A: Mechanism of action and anti-diarrheal efficacy.
Cystic Fibrosis
Inhibition of cystic fibrosis transmembrane conductance regulator by novel interaction with the metabolic sensor AMP-activated protein kinase.
Cystic Fibrosis
Inhibition of cystic fibrosis transmembrane conductance regulator chloride channel currents by arachidonic acid.
Cystic Fibrosis
Inhibition of ENaC by intracellular Cl- in an MDCK clone with high ENaC expression.
Cystic Fibrosis
Inhibition of endocytosis suppresses the nitric oxide-dependent release of Cl- in retinal amacrine cells.
Cystic Fibrosis
Inhibition of enterotoxin-induced porcine colonic secretion by diarylsulfonylureas in vitro.
Cystic Fibrosis
Inhibition of epithelial Na+ currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Inhibition of Heat-Stable Toxin-Induced Intestinal Salt and Water Secretion by a Novel Class of Guanylyl Cyclase C Inhibitors.
Cystic Fibrosis
Inhibition of heterologously expressed cystic fibrosis transmembrane conductance regulator Cl- channels by non-sulphonylurea hypoglycaemic agents.
Cystic Fibrosis
Inhibition of histone deacetylase 6 activity reduces cyst growth in polycystic kidney disease.
Cystic Fibrosis
Inhibition of histone-deacetylase activity rescues inflammatory cystic fibrosis lung disease by modulating innate and adaptive immune responses.
Cystic Fibrosis
Inhibition of IL-8 release from CFTR-deficient lung epithelial cells following pre-treatment with fenretinide.
Cystic Fibrosis
Inhibition of matrix metalloproteinase MMP-2 activates chloride current in human airway epithelial cells.
Cystic Fibrosis
Inhibition of Na+ /H+ exchanger isoform 3 improves gut fluidity and alkalinity in cystic fibrosis transmembrane conductance regulator-deficient and F508del mutant mice.
Cystic Fibrosis
Inhibition of Na+,K+-ATPase by interferon gamma down-regulates intestinal epithelial transport and barrier function.
Cystic Fibrosis
Inhibition of Na+-K+ pump and L-type Ca2+ channel by glibenclamide in Guinea pig ventricular myocytes.
Cystic Fibrosis
Inhibition of neutral sodium absorption by a prostaglandin analogue in patients with cystic fibrosis.
Cystic Fibrosis
Inhibition of NFkappaB by the natural product Withaferin A in cellular models of Cystic Fibrosis inflammation.
Cystic Fibrosis
Inhibition of phosphatidylinositol 3-kinase does not alter forskolin-stimulated Cl(-) secretion by T84 cells.
Cystic Fibrosis
Inhibition of the cystic fibrosis transmembrane conductance regulator by ATP-sensitive K+ channel regulators.
Cystic Fibrosis
Inhibition of volume-regulated and calcium-activated chloride channels by the antimalarial mefloquine.
Cystic Fibrosis
Inhibition of volume-regulated anion channels by expression of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Inhibitors of ATP-binding cassette transporters suppress interleukin-12 p40 production and major histocompatibility complex II up-regulation in macrophages.
Cystic Fibrosis
Inhibitory activities of curzerenone, curdione, furanodienone, curcumol and germacrone on Ca2+-activated chloride channels.
Cystic Fibrosis
Inhibitory effects of glibenclamide on cystic fibrosis transmembrane regulator, swelling-activated, and Ca(2+)-activated Cl- channels in mammalian cardiac myocytes.
Cystic Fibrosis
Inhibitory effects of tumor necrosis factor-alpha on cationic lipid-mediated gene delivery to airway cells in vitro.
Cystic Fibrosis
Inhibitory regulation of cystic fibrosis transmembrane conductance regulator anion-transporting activities by Shank2.
Cystic Fibrosis
Initial evaluation of a biochemical cystic fibrosis newborn screening by sequential analysis of immunoreactive trypsinogen and pancreatitis-associated protein (IRT/PAP) as a strategy that does not involve DNA testing in a Northern European population.
Cystic Fibrosis
Innate immunity and chronic rhinosinusitis: What we have learned from animal models.
Cystic Fibrosis
Innovating cystic fibrosis clinical trial designs in an era of successful standard of care therapies.
Cystic Fibrosis
Insertion of an arginine residue into the transmembrane segments corrects protein misfolding.
Cystic Fibrosis
Insight into cystic fibrosis by structural modelling of CFTR first nucleotide binding fold (NBF1).
Cystic Fibrosis
Insights Into a Possible Influence on Gut Microbiota and Intestinal Barrier Function During Chronic Exposure of Mice to Imazalil.
Cystic Fibrosis
Insights Into Patient Variability During Ivacaftor-Lumacaftor Therapy in Cystic Fibrosis.
Cystic Fibrosis
Insights into the mechanisms underlying CFTR channel activity, the molecular basis for cystic fibrosis and strategies for therapy.
Cystic Fibrosis
Insulin-like growth factor 1 (IGF-1) enhances the protein expression of CFTR.
Cystic Fibrosis
Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis.
Cystic Fibrosis
Integrative expression analysis identifies a novel interplay between CFTR and linc-SUMF1-2 that involves CF-associated gene dysregulation.
Cystic Fibrosis
Interaction among variants in the SLC gene family (SLC6A14, SLC26A9, SLC11A1, and SLC9A3) and CFTR mutations with clinical markers of cystic fibrosis.
Cystic Fibrosis
Interaction between 2 extracellular loops influences the activity of the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Interaction between calcium-activated chloride channels and the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Interaction between cystic fibrosis transmembrane conductance regulator and outwardly rectified chloride channels.
Cystic Fibrosis
Interaction between permeation and gating in a putative pore domain mutant in the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Interaction of intestinal and pancreatic transcription factors in the regulation of CFTR gene expression.
Cystic Fibrosis
Interaction of nucleotides with membrane-associated cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Interaction of the protein phosphatase 2A with the regulatory domain of the cystic fibrosis transmembrane conductance regulator channel.
Cystic Fibrosis
Interactions between ABCC4/MRP4 and ABCC7/CFTR in human airway epithelial cells in lung health and disease.
Cystic Fibrosis
Interactions between impermeant blocking ions in the cystic fibrosis transmembrane conductance regulator chloride channel pore: evidence for anion-induced conformational changes.
Cystic Fibrosis
Interactions between permeant and blocking anions inside the CFTR chloride channel pore.
Cystic Fibrosis
Interdependency of beta-adrenergic receptors and CFTR in regulation of alveolar active Na+ transport.
Cystic Fibrosis
Interest of colchicine for the treatment of cystic fibrosis patients. Preliminary report.
Cystic Fibrosis
Interference with the formation of the epididymal microenvironment--a new strategy for male contraception?
Cystic Fibrosis
Interferon regulatory factor 8 regulates expression of acid ceramidase and infection susceptibility in cystic fibrosis.
Cystic Fibrosis
Interhelical packing in detergent micelles. Folding of a cystic fibrosis transmembrane conductance regulator construct.
Cystic Fibrosis
Interleukin 2 modulates ion secretion and cell proliferation in cultured human small intestinal enterocytes.
Cystic Fibrosis
Interleukin 8 secretion from monocytes of subjects heterozygous for the deltaF508 cystic fibrosis transmembrane conductance regulator gene mutation is altered.
Cystic Fibrosis
Interleukin-13 (IL-13)/IL-13 Receptor {alpha}1 (IL-13R{alpha}1) Signaling Regulates Intestinal Epithelial Cystic Fibrosis Transmembrane Conductance Regulator Channel-dependent Cl- Secretion.
Cystic Fibrosis
Interleukin-17A induces bicarbonate secretion in normal human bronchial epithelial cells.
Cystic Fibrosis
Interleukin-1beta differentially regulates beta2 adrenoreceptor and prostaglandin E2-mediated cAMP accumulation and chloride efflux from Calu-3 bronchial epithelial cells. Role of receptor changes, adenylyl cyclase, cyclo-oxygenase 2, and protein kinase A.
Cystic Fibrosis
Interleukin-4 and interleukin-13 differentially regulate epithelial chloride secretion.
Cystic Fibrosis
Interleukin-8 production by cystic fibrosis nasal epithelial cells after tumor necrosis factor-alpha and respiratory syncytial virus stimulation.
Cystic Fibrosis
Intermolecular interaction between R domains of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Interplay between cystic fibrosis transmembrane regulator and gap junction channels made of connexins 45, 40, 32 and 50 expressed in oocytes.
Cystic Fibrosis
Interplay between ER exit code and domain conformation in CFTR misprocessing and rescue.
Cystic Fibrosis
Interplay between Inhibitory Ferric and Stimulatory Curcumin Regulates Phosphorylation-Dependent Human Cystic Fibrosis Transmembrane Conductance Regulator and ?F508 Activity.
Cystic Fibrosis
Interregulation of proton-gated Na(+) channel 3 and cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Interrelationship between the Na+/glucose cotransporter and CFTR in Caco-2 cells: relevance to cystic fibrosis.
Cystic Fibrosis
Interruption of transmembrane signaling as a novel antisecretory strategy to treat enterotoxigenic diarrhea.
Cystic Fibrosis
Interventions for preventing and managing advanced liver disease in cystic fibrosis.
Cystic Fibrosis
Intestinal anion exchanger down-regulated in adenoma (DRA) is inhibited by intracellular calcium.
Cystic Fibrosis
Intestinal current measurement and nasal potential difference to make a diagnosis of cases with inconclusive CFTR genetics and sweat test.
Cystic Fibrosis
Intestinal Enteroids Model Guanylate Cyclase C-Dependent Secretion Induced by Heat-Stable Enterotoxins.
Cystic Fibrosis
Intestinal organoids and personalized medicine in cystic fibrosis: a successful patient-oriented research collaboration.
Cystic Fibrosis
Intestinal pH and Gastrointestinal Transit Profiles in Cystic Fibrosis Patients Measured by Wireless Motility Capsule.
Cystic Fibrosis
Intestinal physiology and pathology in gene-targeted mouse models of cystic fibrosis.
Cystic Fibrosis
Intracellular cyclic AMP inhibits native and recombinant volume-regulated chloride channels from mammalian heart.
Cystic Fibrosis
Intracellular cysteines of the cystic fibrosis transmembrane conductance regulator (CFTR) modulate channel gating.
Cystic Fibrosis
Intracellular loop between transmembrane segments IV and V of cystic fibrosis transmembrane conductance regulator is involved in regulation of chloride channel conductance state.
Cystic Fibrosis
Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins.
Cystic Fibrosis
Intrathoracic nontuberculous mycobacterial infections in otherwise healthy children.
Cystic Fibrosis
Intrinsic anion channel activity of the recombinant first nucleotide binding fold domain of the cystic fibrosis transmembrane regulator protein.
Cystic Fibrosis
Introduction to PCR/OLA/SCS, a multiplex DNA test, and its application to cystic fibrosis.
Cystic Fibrosis
Intron-8 polythymidine sequence in Australasian individuals with CF mutations R117H and R117C.
Cystic Fibrosis
Intronic enhancers coordinate epithelial-specific looping of the active CFTR locus.
Cystic Fibrosis
Investigating Alternative Transport of Integral Plasma Membrane Proteins from the ER to the Golgi: Lessons from the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Cystic Fibrosis
Involvement of AMP-activated Protein Kinase (AMPK) in Regulation of Cell Membrane Potential in a Gastric Cancer Cell Line.
Cystic Fibrosis
Involvement of CFTR in oviductal HCO3- secretion and its effect on soluble adenylate cyclase-dependent early embryo development.
Cystic Fibrosis
Involvement of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm.
Cystic Fibrosis
Involvement of cystic fibrosis transmembrane conductance regulator (CFTR) in the pathogenesis of hydrosalpinx induced by Chlamydia trachomatis infection.
Cystic Fibrosis
Involvement of cystic fibrosis transmembrane conductance regulator in infection-induced edema.
Cystic Fibrosis
Involvement of cystic fibrosis transmembrane conductance regulator in mouse sperm capacitation.
Cystic Fibrosis
Involvement of F1296 and N1303 of CFTR in induced-fit conformational change in response to ATP binding at NBD2.
Cystic Fibrosis
Involvement of G protein betagamma-subunits in diverse signaling induced by G(i/o)-coupled receptors: study using the Xenopus oocyte expression system.
Cystic Fibrosis
Involvement of hepatocyte nuclear factor 3 in endoderm differentiation of embryonic stem cells.
Cystic Fibrosis
Involvement of phosphatidylinositol 3-kinase in cAMP- and cGMP-induced duodenal epithelial CFTR activation in mice.
Cystic Fibrosis
Involvement of the anion exchanger SLC26A6 in prostaglandin E2- but not forskolin-stimulated duodenal HCO3- secretion.
Cystic Fibrosis
Involvement of the Cdc42 Pathway in CFTR Post-Translational Turnover and in Its Plasma Membrane Stability in Airway Epithelial Cells.
Cystic Fibrosis
Involvement of the cystic fibrosis transmembrane conductance regulator in the acidosis-induced efflux of ATP from rat skeletal muscle.
Cystic Fibrosis
Involvement of the heterodimeric interface region of the nucleotide binding domain-2 (NBD2) in the CFTR quaternary structure and membrane stability.
Cystic Fibrosis
Ion channels in the endometrium: regulation of endometrial receptivity and embryo implantation.
Cystic Fibrosis
Ion Channels in The Pathogenesis of Endometriosis: A Cutting-Edge Point of View.
Cystic Fibrosis
Ion transport mechanisms for smoke inhalation-injured airway epithelial barrier.
Cystic Fibrosis
ION TRANSPORT MECHANISMS LINKED TO BICARBONATE SECRETION IN THE ESOPHAGEAL SUBMUCOSAL GLANDS.
Cystic Fibrosis
Ionic mechanisms of cardiac cell swelling induced by blocking Na+/K+ pump as revealed by experiments and simulation.
Cystic Fibrosis
Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells.
Cystic Fibrosis
Iontophoretic beta-adrenergic stimulation of human sweat glands: possible assay for cystic fibrosis transmembrane conductance regulator activity in vivo.
Cystic Fibrosis
iPSC-Derived Intestinal Organoids from Cystic Fibrosis Patients Acquire CFTR Activity upon TALEN-Mediated Repair of the p.F508del Mutation.
Cystic Fibrosis
Irbit Mediates Synergy Between Ca2+ and cAMP Signaling Pathways During Epithelial Transport in Mice.
Cystic Fibrosis
Iron chelation as novel treatment for lung inflammation in cystic fibrosis.
Cystic Fibrosis
Iron Homeostasis and Inflammatory Status in Mice Deficient for the Cystic Fibrosis Transmembrane Regulator.
Cystic Fibrosis
Is acute recurrent pancreatitis in children a precursor of chronic pancreatitis? A long-term follow-up study of 93 cases.
Cystic Fibrosis
Is CFTR an exchanger?: Regulation of HCO3-Transport and extracellular pH by CFTR.
Cystic Fibrosis
Is congenital bilateral absence of vas deferens a primary form of cystic fibrosis? Analyses of the CFTR gene in 67 patients.
Cystic Fibrosis
Is intracytoplasmic sperm injection itself an indication to perform preimplantation genetic diagnosis (PGD)? About PGD, invasive prenatal diagnosis and genetic sonography.
Cystic Fibrosis
Is It Go or NO Go for S-Nitrosylation Modification-Based Therapies of Cystic Fibrosis Transmembrane Regulator Trafficking?
Cystic Fibrosis
Is the ENaC Dysregulation in CF an Effect of Protein-Lipid Interaction in the Membranes?
Cystic Fibrosis
Is vascular endothelial growth factor (VEGF) the main mediator in ovarian hyperstimulation syndrome (OHSS)?
Cystic Fibrosis
Ischemia-induced enhancement of CFTR expression on the plasma membrane in neonatal rat ventricular myocytes.
Cystic Fibrosis
Islet Function in the Pathogenesis of Cystic Fibrosis-Related Diabetes Mellitus.
Cystic Fibrosis
Isobutylmethylxanthine fails to stimulate chloride secretion in cystic fibrosis airway epithelia.
Cystic Fibrosis
Isoflavone genistein inhibits estrogen-induced chloride and bicarbonate secretory mechanisms in the uterus in rats.
Cystic Fibrosis
Isoforms of SLC26A6 mediate anion transport and have functional PDZ interaction domains.
Cystic Fibrosis
Isogenic cell models of cystic fibrosis-causing variants in natively expressing pulmonary epithelial cells.
Cystic Fibrosis
Isolated elevated sweat chloride concentrations in the presence of the rare mutation S1455X: an extremely mild form of CFTR dysfunction.
Cystic Fibrosis
Isolated idiopathic chronic pancreatitis associated with a compound heterozygosity for two mutations of the CFTR gene.
Cystic Fibrosis
Isolation and characterization of a differentiation-dependent gene in the human colonic cell line HT29-18.
Cystic Fibrosis
Isolation and characterization of rat cholangiocyte vesicles enriched in apical or basolateral plasma membrane domains.
Cystic Fibrosis
Isolation of CFTR and TMEM16A inhibitors from Neorautanenia mitis (A. Rich) Verdcourt: Potential lead compounds for treatment of secretory diarrhea.
Cystic Fibrosis
Isotype-specific activation of cystic fibrosis transmembrane conductance regulator-chloride channels by cGMP-dependent protein kinase II.
Cystic Fibrosis
It's not all about inhaled treatment: challenges with oral therapy in paediatric respiratory medicine.
Cystic Fibrosis
Ivacaftor and sinonasal pathology in a cystic fibrosis patient with genotype deltaF508/S1215N.
Cystic Fibrosis
Ivacaftor for the p.Ser549Arg (S549R) gating mutation - The Israeli experience.
Cystic Fibrosis
Ivacaftor for the treatment of cystic fibrosis in children under six years of age.
Cystic Fibrosis
Ivacaftor improves appearance of sinus disease on computerised tomography in cystic fibrosis patients with G551D mutation.
Cystic Fibrosis
Ivacaftor improves rhinologic, psychologic, and sleep-related quality of life in G551D cystic fibrosis patients.
Cystic Fibrosis
Ivacaftor in a young boy with the rare gating mutation S549R--use of lung clearance index to track progress: a case report.
Cystic Fibrosis
Ivacaftor in People with Cystic Fibrosis and a 3849+10kb C?T or D1152H Residual Function Mutation.
Cystic Fibrosis
IVACAFTOR restores FGF19 regulated bile acid homeostasis in cystic fibrosis patients with an S1251N or a G551D gating mutation.
Cystic Fibrosis
Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation.
Cystic Fibrosis
Ivacaftor, a Cystic Fibrosis Transmembrane Conductance Regulator Potentiator, Enhances Ciprofloxacin Activity Against Pseudomonas aeruginosa.
Cystic Fibrosis
Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis.
Cystic Fibrosis
Ivacaftor: the first therapy acting on the primary cause of cystic fibrosis.
Cystic Fibrosis
IVS8-5T Allele of CFTR is the Risk Factor in Chronic Pancreatitis, Especially in Idiopathic Chronic Pancreatitis.
Cystic Fibrosis
Janus kinase 2 (JAK2) regulates prolactin-mediated chloride transport in mouse mammary epithelial cells through tyrosine phosphorylation of Na+-K+-2Cl- cotransporter.
Cystic Fibrosis
KCa3.1 potassium channels are critical for cAMP-dependent chloride secretion and cyst growth in autosomal-dominant polycystic kidney disease.
Cystic Fibrosis
Keratin K18 increases cystic fibrosis transmembrane conductance regulator (CFTR) surface expression by binding to its C-terminal hydrophobic patch.
Cystic Fibrosis
Kinin-stimulated chloride secretion in mouse colon requires the participation of CFTR chloride channels.
Cystic Fibrosis
Knockdown of CFTR enhances sensitivity of prostate cancer cells to cisplatin via inhibition of autophagy.
Cystic Fibrosis
Knockout mice reveal key roles for claudin 18 in alveolar barrier properties and fluid homeostasis.
Cystic Fibrosis
Knockout mouse models for intestinal electrolyte transporters and regulatory PDZ adaptors: new insights into cystic fibrosis, secretory diarrhoea and fructose-induced hypertension.
Cystic Fibrosis
l-Methionine anti-biofilm activity against Pseudomonas aeruginosa is enhanced by the cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor.
Cystic Fibrosis
L206W mutation of the cystic fibrosis gene, relatively frequent in French Canadians, is associated with atypical presentations of cystic fibrosis.
Cystic Fibrosis
Laboratory and clinical studies in support of cystic fibrosis gene therapy using pCMV-CFTR-DOTAP.
Cystic Fibrosis
Laboratory reporting on the clinical spectrum of CFTR p.Arg117His: Still room for improvement.
Cystic Fibrosis
Lack of association of common cystic fibrosis transmembrane conductance regulator gene mutations with primary sclerosing cholangitis.
Cystic Fibrosis
Lack of correlation between CFTR expression, CFTR Cl- currents, amiloride-sensitive Na+ conductance, and cystic fibrosis phenotype.
Cystic Fibrosis
Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report.
Cystic Fibrosis
Lack of cystic fibrosis transmembrane conductance regulator disrupts fetal airway development in pigs.
Cystic Fibrosis
Lack of cystic fibrosis transmembrane regulator-type chloride current in pediatric human atrial myocytes.
Cystic Fibrosis
Lack of effect of delta F508 mutation on aerobic capacity in patients with cystic fibrosis.
Cystic Fibrosis
Lack of involvement of G proteins in the activation of cardiac CFTR Cl- current by genistein.
Cystic Fibrosis
Lactobacillus casei protects dextran sodium sulfate- or rapamycin-induced colonic inflammation in the mouse.
Cystic Fibrosis
Laminin 411 and 511 promote the cholangiocyte differentiation of human induced pluripotent stem cells.
Cystic Fibrosis
Learning from each other: ABC transporter regulation by protein phosphorylation in plant and mammalian systems.
Cystic Fibrosis
Lectin conjugates as potent, nonabsorbable CFTR inhibitors for reducing intestinal fluid secretion in cholera.
Cystic Fibrosis
Lentiviral Vectors for the Treatment and Prevention of Cystic Fibrosis Lung Disease.
Cystic Fibrosis
Lentiviral vectors transduce lung stem cells without disrupting plasticity.
Cystic Fibrosis
Leporizines a-C: epithiodiketopiperazines isolated from an Aspergillus species.
Cystic Fibrosis
Levamisole inhibits intestinal Cl- secretion via basolateral K+ channel blockade.
Cystic Fibrosis
Levels of pro- and anti-inflammatory cytokines in cystic fibrosis patients with or without gingivitis.
Cystic Fibrosis
Ligand binding to a remote site thermodynamically corrects the F508del mutation in the human cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Limited restoration of cystic fibrosis lung epithelium in vivo with adult bone marrow-derived cells.
Cystic Fibrosis
Linaclotide activates guanylate cyclase-C/cGMP/protein kinase-II-dependent trafficking of CFTR in the intestine.
Cystic Fibrosis
Linaclotide Improves Gastrointestinal Transit in Cystic Fibrosis Mice by Inhibiting Sodium/Hydrogen Exchanger 3.
Cystic Fibrosis
Linaclotide-a Novel Secretagogue in The Treatment of Irritable Bowel Syndrome with Constipation and Chronic Idiopathic Constipation.
Cystic Fibrosis
Linaclotide: a new option for the treatment of irritable bowel syndrome with constipation and chronic idiopathic constipation in adults.
Cystic Fibrosis
Linear-After-The-Exponential (LATE)-PCR: primer design criteria for high yields of specific single-stranded DNA and improved real-time detection.
Cystic Fibrosis
Link between CFTR mutations and ABPA: a systematic review and meta-analysis.
Cystic Fibrosis
Linkage analysis of candidate loci in autosomal dominant myotonia congenita.
Cystic Fibrosis
Linkage disequilibrium between the M470V variant and the IVS8 polyT alleles of the CFTR gene in CBAVD.
Cystic Fibrosis
Linkage to Gaucher mutations in the Ashkenazi population: effect of drift on decay of linkage disequilibrium and evidence for heterozygote selection.
Cystic Fibrosis
Linoleic acid supplementation results in increased arachidonic acid and eicosanoid production in CF airway cells and in cftr-/- transgenic mice.
Cystic Fibrosis
Lipid Nanoparticle-Delivered Chemically Modified mRNA Restores Chloride Secretion in Cystic Fibrosis.
Cystic Fibrosis
Lipid Peroxidation Drives Renal Cyst Growth In Vitro through Activation of TMEM16A.
Cystic Fibrosis
Lipophilicity of the Cystic Fibrosis Drug, Ivacaftor (VX-770), and Its Destabilizing Effect on the Major CF-causing Mutation: F508del.
Cystic Fibrosis
Liposomes loaded with bioactive lipids enhance antibacterial innate immunity irrespective of drug resistance.
Cystic Fibrosis
Liposomes Loaded With Phosphatidylinositol 5-Phosphate Improve the Antimicrobial Response to Pseudomonas aeruginosa in Impaired Macrophages From Cystic Fibrosis Patients and Limit Airway Inflammatory Response.
Cystic Fibrosis
Listeria monocytogenes exploits cystic fibrosis transmembrane conductance regulator (CFTR) to escape the phagosome.
Cystic Fibrosis
Listeria monocytogenes mediated CFTR transgene transfer to mammalian cells.
Cystic Fibrosis
Lithocholic acid attenuates cAMP-dependent Cl- secretion in human colonic epithelial T84 cells.
Cystic Fibrosis
Little Cigars are More Toxic than Cigarettes and Uniquely Change the Airway Gene and Protein Expression.
Cystic Fibrosis
Liver disease in pediatric patients with cystic fibrosis is associated with glutathione S-transferase P1 polymorphism.
Cystic Fibrosis
Liver expression in cystic fibrosis could be modulated by genetic factors different from the cystic fibrosis transmembrane regulator genotype.
Cystic Fibrosis
Liver X receptor ? regulates bile volume and the expression of aquaporins and cystic fibrosis transmembrane conductance regulator in the gallbladder.
Cystic Fibrosis
LMTK2 Mediated Phosphorylation Regulates CFTR Endocytosis in Human Airway Epithelial Cells.
Cystic Fibrosis
LncRNA and transcriptomic analysis of fetal membrane reveal potential targets involved in oligohydramnios.
Cystic Fibrosis
Local modulation of Cystic Fibrosis Conductance Regulator: cytoskeleton and compartmentalised cAMP signalling.
Cystic Fibrosis
Local regulation of cystic fibrosis transmembrane regulator and epithelial sodium channel in airway epithelium.
Cystic Fibrosis
Localised mutagenesis of the fts YEX operon: conditionally lethal missense substitutions in the FtsE cell division protein of Escherichia coli are similar to those found in the cystic fibrosis transmembrane conductance regulator protein (CFTR) of human patients.
Cystic Fibrosis
Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding.
Cystic Fibrosis
Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia.
Cystic Fibrosis
Localization of cystic fibrosis transmembrane conductance regulator in epithelial cells of nasal polyps and postoperative polypoid mucosae.
Cystic Fibrosis
Localization of cystic fibrosis transmembrane conductance regulator mRNA in human fetal lung tissue by in situ hybridization.
Cystic Fibrosis
Localization of cystic fibrosis transmembrane conductance regulator mRNA in the human gastrointestinal tract by in situ hybridization.
Cystic Fibrosis
Localization of cystic fibrosis transmembrane conductance regulator signaling complexes in human salivary gland striated duct cells.
Cystic Fibrosis
Localization of cystic fibrosis transmembrane conductance regulator to lipid rafts of epithelial cells is required for Pseudomonas aeruginosa-induced cellular activation.
Cystic Fibrosis
Localization of sequences within the C-terminal domain of the cystic fibrosis transmembrane conductance regulator which impact maturation and stability.
Cystic Fibrosis
Localization of the BiP molecular chaperone with respect to endoplasmic reticulum foci containing the cystic fibrosis transmembrane conductance regulator in yeast.
Cystic Fibrosis
Localization of the cystic fibrosis transmembrane conductance regulator (Cftr) to mouse chromosome 6.
Cystic Fibrosis
Localization of the cystic fibrosis transmembrane conductance regulator in airway secretory glands.
Cystic Fibrosis
Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells.
Cystic Fibrosis
Localization of the cystic fibrosis transmembrane conductance regulator in pancreas.
Cystic Fibrosis
Localization of the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) in the rat to chromosome 4 and implications for the evolution of mammalian chromosomes.
Cystic Fibrosis
Localization studies of rare missense mutations in cystic fibrosis transmembrane conductance regulator (CFTR) facilitate interpretation of genotype-phenotype relationships.
Cystic Fibrosis
Locating a Plausible Binding Site for an Open Channel Blocker, GlyH-101, in the Pore of the Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
Locating the anion-selectivity filter of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel.
Cystic Fibrosis
Location of a common inhibitor binding site in the cytoplasmic vestibule of the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Cystic Fibrosis
Location of a permeant anion binding site in the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Cystic Fibrosis
Long Chain Polyunsaturated Fatty acids in Granulocytes, Mononuclear Cells and Red Blood Cells in Patients with Cystic Fibrosis: Relation to Liver Disease.
Cystic Fibrosis
Long Non-coding RNA BGas Regulates the Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation.
Cystic Fibrosis
Long-range (17.7 kb) allele-specific polymerase chain reaction method for direct haplotyping of R117H and IVS-8 mutations of the cystic fibrosis transmembrane regulator gene.
Cystic Fibrosis
Long-range coupling between the extracellular gates and the intracellular ATP binding domains of multidrug resistance protein pumps and cystic fibrosis transmembrane conductance regulator channels.
Cystic Fibrosis
Long-term cAMP activation of Na(+)-K(+)-2Cl- cotransporter activity in HT-29 human adenocarcinoma cells.
Cystic Fibrosis
Long-term docosahexaenoic acid therapy in a congenic murine model of cystic fibrosis.
Cystic Fibrosis
Long-Term Ivacaftor in People Aged 6 Years and Older with Cystic Fibrosis with Ivacaftor-Responsive Mutations.
Cystic Fibrosis
Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST).
Cystic Fibrosis
Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study.
Cystic Fibrosis
Long-term therapeutic and reporter gene expression in lentiviral vector treated cystic fibrosis mice.
Cystic Fibrosis
Long-wavelength iodide-sensitive fluorescent indicators for measurement of functional CFTR expression in cells.
Cystic Fibrosis
Longevity and plasticity of CFTR provide an argument for noncanonical SNP organization in hominid DNA.
Cystic Fibrosis
Lonidamine and analogue AF2785 block the cyclic adenosine 3', 5'-monophosphate-activated chloride current and chloride secretion in the rat epididymis.
Cystic Fibrosis
Loop diuretics are open-channel blockers of the cystic fibrosis transmembrane conductance regulator with distinct kinetics.
Cystic Fibrosis
Loop Sequence Dictates the Secondary Structure of a Human Membrane Protein Hairpin.
Cystic Fibrosis
Losartan reduces cigarette smoke-induced airway inflammation and mucus hypersecretion.
Cystic Fibrosis
Loss of Ca-mediated ion transport during colitis correlates with reduced ion transport responses to a Ca-activated K channel opener.
Cystic Fibrosis
Loss of CFTR Affects Biliary Epithelium Innate Immunity and Causes TLR4-NF-?B-Mediated Inflammatory Response in Mice.
Cystic Fibrosis
Loss of Cftr function exacerbates the phenotype of Na+ hyperabsorption in murine airways.
Cystic Fibrosis
Loss of cystic fibrosis transmembrane conductance regulator function enhances activation of p38 and ERK MAPKs, increasing interleukin-6 synthesis in airway epithelial cells exposed to Pseudomonas aeruginosa.
Cystic Fibrosis
Loss of cystic fibrosis transmembrane conductance regulator impairs lung endothelial cell barrier function and increases susceptibility to microvascular damage from cigarette smoke.
Cystic Fibrosis
Loss of Cystic Fibrosis Transmembrane Regulator Impairs Intestinal Oxalate Secretion.
Cystic Fibrosis
Loss of downregulated in adenoma (DRA) impairs mucosal HCO(3) (-) secretion in murine ileocolonic inflammation.
Cystic Fibrosis
Loss of PKD1 and PKD2 share common effects on intracellular Ca2+ signaling.
Cystic Fibrosis
Loss of Slc26a9 anion transporter alters intestinal electrolyte and HCO3 (-) transport and reduces survival in CFTR-deficient mice.
Cystic Fibrosis
Loss of SLC9A3 decrease CFTR protein and causes obstructed azoospermia in mice.
Cystic Fibrosis
Loss of the normal epicardial to endocardial gradient of cftr mRNA expression in the hypertrophied rabbit left ventricle.
Cystic Fibrosis
Low abundance of sweat duct Cl- channel CFTR in both healthy and cystic fibrosis athletes with exceptionally salty sweat during exercise.
Cystic Fibrosis
Low Beta-Adrenergic Sweat Responses in Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome Children.
Cystic Fibrosis
Low sphingosine-1-phosphate impairs lung dendritic cells in cystic fibrosis.
Cystic Fibrosis
Low temperature and chemical rescue affect molecular proximity of DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) and epithelial sodium channel (ENaC).
Cystic Fibrosis
Lower Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Promotes the Proliferation and Migration of Endometrial Carcinoma.
Cystic Fibrosis
Lower exhaled nitric oxide in infants with Cystic Fibrosis compared to healthy controls.
Cystic Fibrosis
Lubiprostone activates non-CFTR-dependent respiratory epithelial chloride secretion in cystic fibrosis mice.
Cystic Fibrosis
Lubiprostone Targets Prostanoid Signaling and Promotes Ion Transporter Trafficking, Mucus Exocytosis, and Contractility.
Cystic Fibrosis
Lumacaftor alone and combined with ivacaftor: preclinical and clinical trial experience of F508del CFTR correction.
Cystic Fibrosis
Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR.
Cystic Fibrosis
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.
Cystic Fibrosis
Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion.
Cystic Fibrosis
Lumacaftor/ivacaftor therapy is associated with reduced hepatic steatosis in cystic fibrosis patients.
Cystic Fibrosis
Lumacaftor/ivacaftor, a novel agent for the treatment of cystic fibrosis patients who are homozygous for the F580del CFTR mutation.
Cystic Fibrosis
Luminal hydrochloric acid stimulates rapid transepithelial ion fluxes in rodent esophageal stratified squamous epithelium.
Cystic Fibrosis
Luminally Acting Agents for Constipation Treatment: A Review Based on Literatures and Patents.
Cystic Fibrosis
Luminally active, nonabsorbable CFTR inhibitors as potential therapy to reduce intestinal fluid loss in cholera.
Cystic Fibrosis
Lung arginase expression and activity is increased in cystic fibrosis mouse models.
Cystic Fibrosis
Lung edema clearance: 20 years of progress: invited review: alveolar edema fluid clearance in the injured lung.
Cystic Fibrosis
Lung function changes in relation to menstrual cycle in females with cystic fibrosis.
Cystic Fibrosis
Lung gene therapy with highly compacted DNA nanoparticles that overcome the mucus barrier.
Cystic Fibrosis
Lung symptoms in pseudohypoaldosteronism type 1 are associated with deficiency of the alpha-subunit of the epithelial sodium channel.
Cystic Fibrosis
Luteolin reduces fluid hypersecretion by inhibiting TMEM16A in interleukin-4 treated Calu-3 airway epithelial cells.
Cystic Fibrosis
Lymphocyte CFTR promotes epithelial bicarbonate secretion for bacterial killing.
Cystic Fibrosis
Lysophosphatidic acid inhibits cholera toxin-induced secretory diarrhea through CFTR-dependent protein interactions.
Cystic Fibrosis
Lysophosphatidylglycerol: a novel effective detergent for solubilizing and purifying the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Lysosomal Reacidification Ameliorates Vinyl Carbamate-Induced Toxicity and Disruption on Lysosomal pH.
Cystic Fibrosis
Macromolecular complexes of cystic fibrosis transmembrane conductance regulator and its interacting partners.
Cystic Fibrosis
Magnetomotive optical coherence elastography for relating lung structure and function in Cystic Fibrosis.
Cystic Fibrosis
Maintaining low Ca2+ level in the endoplasmic reticulum restores abnormal endogenous F508del-CFTR trafficking in airway epithelial cells.
Cystic Fibrosis
Male partners of infertile couples with congenital unilateral absence of the vas deferens are mainly non-azoospermic.
Cystic Fibrosis
Mammalian osmolytes and S-nitrosoglutathione promote Delta F508 cystic fibrosis transmembrane conductance regulator (CFTR) protein maturation and function.
Cystic Fibrosis
Management of male infertility due to congenital bilateral absence of vas deferens should not ignore the diagnosis of cystic fibrosis.
Cystic Fibrosis
Managing Fungal Infections in Cystic Fibrosis Patients: Challenges in Clinical Practice.
Cystic Fibrosis
Manifestation of cystic fibrosis transmembrane regulator (CFTR) in hepatic ductal structures and renal tubules of female rats with experimental cholestasis of pregnancy.
Cystic Fibrosis
Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis.
Cystic Fibrosis
Mapping of cystic fibrosis transmembrane conductance regulator membrane topology by glycosylation site insertion.
Cystic Fibrosis
Mapping of genetic factors influencing the weight of cystic fibrosis knockout mice.
Cystic Fibrosis
Markedly elevated neonatal immunoreactive trypsinogen levels in the absence of cystic fibrosis gene mutations is not an indication for further testing.
Cystic Fibrosis
Mashiningan Improves Opioid-Induced Constipation in Rats by Activating Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel.
Cystic Fibrosis
MAST205 Competes with CAL for Binding to CFTR to Regulate CFTR-mediated Fluid Transport.
Cystic Fibrosis
Maternal and fetal outcomes following elexacaftor-tezacaftor-ivacaftor use during pregnancy and lactation.
Cystic Fibrosis
Matrine in association with FD?2 stimulates F508del?cystic fibrosis transmembrane conductance regulator activity in the presence of corrector VX809.
Cystic Fibrosis
Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2.
Cystic Fibrosis
Maxi K+ channels co-localised with CFTR in the apical membrane of an exocrine gland acinus: possible involvement in secretion.
Cystic Fibrosis
Maxillary sinusitis as a surrogate model for CF gene therapy clinical trials in patients with antrostomies.
Cystic Fibrosis
Maximization of the rate of chloride conduction in the CFTR channel pore by ion-ion interactions.
Cystic Fibrosis
Measurement of cystic fibrosis transmembrane conductance regulator activity using fluorescence spectrophotometry.
Cystic Fibrosis
Measurement of Multi Ion Transport through Human Bronchial Epithelial Cell Line Provides an Insight into the Mechanism of Defective Water Transport in Cystic Fibrosis.
Cystic Fibrosis
Mechanical Properties of Human Bronchial Epithelial Cells Expressing Wt- and Mutant CFTR.
Cystic Fibrosis
Mechanism and cellular applications of a green fluorescent protein-based halide sensor.
Cystic Fibrosis
Mechanism of chloride permeation in the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Mechanism of dysfunction of two nucleotide binding domain mutations in cystic fibrosis transmembrane conductance regulator that are associated with pancreatic sufficiency.
Cystic Fibrosis
Mechanism of enhancement of DNA expression consequent to cointernalization of a replication-deficient adenovirus and unmodified plasmid DNA.
Cystic Fibrosis
Mechanism of G551D-CFTR (cystic fibrosis transmembrane conductance regulator) potentiation by a high affinity ATP analog.
Cystic Fibrosis
Mechanism of glibenclamide inhibition of cystic fibrosis transmembrane conductance regulator Cl- channels expressed in a murine cell line.
Cystic Fibrosis
Mechanism of inhibition of cAMP-dependent epithelial chloride secretion by phorbol esters.
Cystic Fibrosis
Mechanism of substance P-induced liquid secretion across bronchial epithelium.
Cystic Fibrosis
Mechanism-based corrector combination restores ?F508-CFTR folding and function.
Cystic Fibrosis
Mechanism-Based Personalized Medicine for Cystic Fibrosis by Suppressing Pseudo Exon Inclusion.
Cystic Fibrosis
Mechanisms of attenuation of abdominal sepsis induced acute lung injury by ascorbic acid.
Cystic Fibrosis
Mechanisms of Ca2+-stimulated fluid secretion by porcine bronchial submucosal gland serous acinar cells.
Cystic Fibrosis
Mechanisms of cystic fibrosis transmembrane conductance regulator activation by S-nitrosoglutathione.
Cystic Fibrosis
Mechanisms of guanylin action on water and ion absorption at different regions of seawater eel intestine.
Cystic Fibrosis
Mechanisms of the inhibition of epithelial Na(+) channels by CFTR and purinergic stimulation.
Cystic Fibrosis
Mechanistic analysis and significance of sphingomyelinase-mediated decreases in transepithelial CFTR currents in nHBEs.
Cystic Fibrosis
Mechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic Fibrosis.
Cystic Fibrosis
Mechanosensitive activation of CFTR by increased cell volume and hydrostatic pressure but not shear stress.
Cystic Fibrosis
Mechanosensitivity of wild-type and G551D cystic fibrosis transmembrane conductance regulator (CFTR) controls regulatory volume decrease in simple epithelia.
Cystic Fibrosis
Medical devices; immunology and microbiology devices; classification of cystic fibrosis transmembrane conductance regulator gene mutation detection system. Final rule.
Cystic Fibrosis
Melatonin receptor potentiation of cyclic AMP and the cystic fibrosis transmembrane conductance regulator ion channel.
Cystic Fibrosis
Membrane insertion, processing, and topology of cystic fibrosis transmembrane conductance regulator (CFTR) in microsomal membranes.
Cystic Fibrosis
Membrane lateral diffusion and capture of CFTR within transient confinement zones.
Cystic Fibrosis
Membrane protein stability can be compromised by detergent interactions with the extramembranous soluble domains.
Cystic Fibrosis
Membrane targeting of cGMP-dependent protein kinase is required for cystic fibrosis transmembrane conductance regulator Cl- channel activation.
Cystic Fibrosis
Membrane topology and glycosylation of the human multidrug resistance-associated protein.
Cystic Fibrosis
Membrane topology distinguishes a subfamily of the ATP-binding cassette (ABC) transporters.
Cystic Fibrosis
Membrane trafficking of the cystic fibrosis gene product, cystic fibrosis transmembrane conductance regulator, tagged with green fluorescent protein in madin-darby canine kidney cells.
Cystic Fibrosis
Membrane-integration characteristics of two ABC transporters, CFTR and P-glycoprotein.
Cystic Fibrosis
Membrane-specific regulation of Cl- channels by purinergic receptors in rat submandibular gland acinar and duct cells.
Cystic Fibrosis
Mercury toxicity in the shark (Squalus acanthias) rectal gland: apical CFTR chloride channels are inhibited by mercuric chloride.
Cystic Fibrosis
Mesenchymal Stem Cell exosome delivered Zinc Finger Protein activation of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Metabolic Reprograming of Cystic Fibrosis Macrophages via the IRE1? Arm of the Unfolded Protein Response Results in Exacerbated Inflammation.
Cystic Fibrosis
Metabolic Signatures of Cystic Fibrosis Identified in Dried Blood Spots For Newborn Screening Without Carrier Identification.
Cystic Fibrosis
Metabolomic profiling reveals biochemical pathways and biomarkers associated with pathogenesis in cystic fibrosis cells.
Cystic Fibrosis
Metal bridges illuminate transmembrane domain movements during gating of the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Metformin slows liver cyst formation and fibrosis in experimental model of polycystic liver disease.
Cystic Fibrosis
Metformin treatment of diabetes mellitus increases the risk for pancreatitis in patients bearing the CFTR-mutation S573C.
Cystic Fibrosis
Methods for the Use of Retroviral Vectors for Transfer of the CFTR Gene to Airway Epithelium.
Cystic Fibrosis
Methods to monitor cell surface expression and endocytic trafficking of CFTR in polarized epithelial cells.
Cystic Fibrosis
Methylation profile of the promoter CpG islands of 14 "drug-resistance" genes in hepatocellular carcinoma.
Cystic Fibrosis
mGluR1 interacts with cystic fibrosis transmembrane conductance regulator and modulates the secretion of IL-10 in cystic fibrosis peripheral lymphocytes.
Cystic Fibrosis
Microarray analysis reveals the inhibition of intestinal expression of nutrient transporters in piglets infected with porcine epidemic diarrhea virus.
Cystic Fibrosis
Microbial-induced meprin ? cleavage in MUC2 mucin and a functional CFTR channel are required to release anchored small intestinal mucus.
Cystic Fibrosis
Microelectrode measurements of the effects of basolateral adenosine in polarized human intestinal epithelial cells in culture.
Cystic Fibrosis
microRNA regulation of expression of the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
MicroRNA-1246 regulates proliferation, invasion, and differentiation in human vascular smooth muscle cells by targeting cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
MicroRNA-145, Cystic Fibrosis Transmembrane Conductance Regulator, and Transforming Growth Factor-?. An (Un)tangled Regulatory Web.
Cystic Fibrosis
MicroRNA-9 downregulates the ANO1 chloride channel and contributes to cystic fibrosis lung pathology.
Cystic Fibrosis
Microsatellite haplotypes associations with 5 CFTR mutations in "Grande Brière", an isolate located in southern Brittany.
Cystic Fibrosis
Microtubule disruption inhibits AVT-stimulated Cl- secretion but not Na+ reabsorption in A6 cells.
Cystic Fibrosis
Mid-trimester hyperechogenic bowel in a fetus of Japanese origin carrying a new mutation of CFTR gene (L548Q).
Cystic Fibrosis
Mild course of cystic fibrosis associated with heterozygosity for infrequent mutations in the first nucleotide-binding fold of CFTR.
Cystic Fibrosis
MiR-101 and miR-144 regulate the expression of the CFTR chloride channel in the lung.
Cystic Fibrosis
miR-125b/NRF2/HO-1 axis is involved in protection against oxidative stress of cystic fibrosis: A pilot study.
Cystic Fibrosis
miR-16 rescues F508del-CFTR function in native cystic fibrosis epithelial cells.
Cystic Fibrosis
miR-17 overexpression in cystic fibrosis airway epithelial cells decreases interleukin-8 production.
Cystic Fibrosis
Misassembled mutant DeltaF508 CFTR in the distal secretory pathway alters cellular lipid trafficking.
Cystic Fibrosis
Misfolding of the cystic fibrosis transmembrane conductance regulator and disease.
Cystic Fibrosis
Mismatch-targeted transposition of Mu: a new strategy to map genetic polymorphism.
Cystic Fibrosis
Missense mutation within cystic fibrosis transmembrane conductance regulator (CFTR) gene is associated with selected parameters of the frozen-thawed sperm in Holstein-Friesian bulls.
Cystic Fibrosis
Missense mutations in SLC26A8, encoding a sperm-specific activator of CFTR, are associated with human asthenozoospermia.
Cystic Fibrosis
Missense mutations in the cystic fibrosis gene in adult patients with asthma.
Cystic Fibrosis
Missense mutations in transmembrane domains of proteins: phenotypic propensity of polar residues for human disease.
Cystic Fibrosis
Missense variants in CFTR nucleotide-binding domains predict quantitative phenotypes associated with cystic fibrosis disease severity.
Cystic Fibrosis
Missense, nonsense, and neutral mutations define juxtaposed regulatory elements of splicing in cystic fibrosis transmembrane regulator exon 9.
Cystic Fibrosis
Mitochondrial oxidative stress in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice.
Cystic Fibrosis
Mobilization of intracellular Ca2+ and stimulation of cyclic AMP production by kappa opioid receptors expressed in Xenopus oocytes.
Cystic Fibrosis
Model of the cAMP activation of chloride transport by CFTR channel and the mechanism of potentiators.
Cystic Fibrosis
Modeling Cystic Fibrosis Using Pluripotent Stem Cell-Derived Human Pancreatic Ductal Epithelial Cells.
Cystic Fibrosis
Modeling of nucleotide binding domains of ABC transporter proteins based on a F1-ATPase/recA topology: structural model of the nucleotide binding domains of the cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
Modelling the restoration of wild-type dynamic behaviour in DeltaF508-CFTR NBD1 by 8-cyclopentyl-1,3-dipropylxanthine.
Cystic Fibrosis
Modification of nasal epithelial potential differences of individuals with cystic fibrosis consequent to local administration of a normal CFTR cDNA adenovirus gene transfer vector.
Cystic Fibrosis
Modification of transepithelial ion transport in human cultured bronchial epithelial cells by interferon-gamma.
Cystic Fibrosis
Modulating the folding of P-glycoprotein and cystic fibrosis transmembrane conductance regulator truncation mutants with pharmacological chaperones.
Cystic Fibrosis
Modulation of CFTR gene expression in HT-29 cells by extracellular hyperosmolarity.
Cystic Fibrosis
Modulation of Chloride Channel Functions by the Plant Lignan Compounds Kobusin and Eudesmin.
Cystic Fibrosis
Modulation of Cl- secretion by benzimidazolones. II. Coordinate regulation of apical GCl and basolateral GK.
Cystic Fibrosis
Modulation of cystic fibrosis transmembrane conductance regulator activity and genistein binding by cytosolic pH.
Cystic Fibrosis
Modulation of cystic fibrosis transmembrane conductance regulator gene - expression by elevation of intracellular cyclic AMP.
Cystic Fibrosis
Modulation of deltaF508 cystic fibrosis transmembrane regulator trafficking and function with 4-phenylbutyrate and flavonoids.
Cystic Fibrosis
Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor.
Cystic Fibrosis
Modulation of ENaC, CFTR, and iNOS expression in bronchial epithelial cells after stimulation with Staphylococcus epidermidis (94B080) and Staphylococcus aureus (90B083).
Cystic Fibrosis
Modulation of epithelial sodium channel trafficking and function by sodium 4-phenylbutyrate in human nasal epithelial cells.
Cystic Fibrosis
Modulation of Hepatic MRP3/ABCC3 by Xenobiotics and Pathophysiological Conditions: Role in Drug Pharmacokinetics.
Cystic Fibrosis
Modulation of mature cystic fibrosis transmembrane regulator protein by the PDZ domain protein CAL.
Cystic Fibrosis
Modulation of Polycystic Kidney Disease Severity by Phosphodiesterase 1 and 3 Subfamilies.
Cystic Fibrosis
Modulation of protein kinase CK2 activity by fragments of CFTR encompassing F508 may reflect functional links with cystic fibrosis pathogenesis.
Cystic Fibrosis
Modulation of sodium transport in alveolar epithelial cells by estradiol and progesterone.
Cystic Fibrosis
Modulation of Treg function improves adenovirus vector-mediated gene expression in the airway.
Cystic Fibrosis
Modulators of Ion Transport in Nasal Polyps: An in situ Measurement of Short-Circuit Current.
Cystic Fibrosis
Modulators of the Cystic Fibrosis Transmembrane Conductance Regulator Protein.
Cystic Fibrosis
Molecular analysis in Brazilian cystic fibrosis patients reveals five novel mutations.
Cystic Fibrosis
Molecular analysis of 23 exons of the CFTR gene in Brazilian patients leads to the finding of rare cystic fibrosis mutations.
Cystic Fibrosis
Molecular analysis of mutations and polymorphisms in the CFTR gene in male infertility.
Cystic Fibrosis
Molecular analysis of the cystic fibrosis gene reveals a high frequency of the intron 8 splice variant 5T in Egyptian males with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
Molecular analysis of the IVS8-T splice variant 5T and M470V exon 10 missense polymorphism in Iranian males with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
Molecular analysis of the ovine cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Molecular and Functional Analysis of the Large 5' Promoter Region of CFTR Gene Revealed Pathogenic Mutations in CF and CFTR-Related Disorders.
Cystic Fibrosis
Molecular and functional characterization of CBAVD-causing mutations located in CFTR nucleotide-binding domains.
Cystic Fibrosis
Molecular and functional characterization of the cystic fibrosis transmembrane conductance regulator from the Australian common brushtail possum, Trichosurus vulpecula.
Cystic Fibrosis
Molecular and functional heterogeneity of cholangiocytes from rat liver after bile duct ligation.
Cystic Fibrosis
Molecular assembly of cystic fibrosis transmembrane conductance regulator in plasma membrane.
Cystic Fibrosis
Molecular Basis and Differentiation-Associated Alterations of Anion Secretion in Human Duodenal Enteroid Monolayers.
Cystic Fibrosis
Molecular Basis for Fe(III)-Independent Curcumin Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Activity.
Cystic Fibrosis
Molecular basis for the chloride channel activity of cystic fibrosis transmembrane conductance regulator and the consequences of disease-causing mutations.
Cystic Fibrosis
Molecular basis of bicarbonate membrane transport in the male reproductive tract.
Cystic Fibrosis
Molecular basis of cystic fibrosis in Lithuania: incomplete CFTR mutation detection by PCR-based screening protocols.
Cystic Fibrosis
Molecular basis of UG-rich RNA recognition by the human splicing factor TDP-43.
Cystic Fibrosis
Molecular Chaperones as Targets to Circumvent the CFTR Defect in Cystic Fibrosis.
Cystic Fibrosis
Molecular characterization of cystic fibrosis: 16 novel mutations identified by analysis of the whole cystic fibrosis conductance transmembrane regulator (CFTR) coding regions and splice site junctions.
Cystic Fibrosis
Molecular characterization of the cystic fibrosis transmembrane conductance regulator gene in congenital absence of the vas deferens.
Cystic Fibrosis
Molecular cloning and expression of a cyclic AMP-activated chloride conductance regulator: a novel ATP-binding cassette transporter.
Cystic Fibrosis
Molecular cloning and sequence analysis of the murine cDNA for the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Molecular cloning, expression analysis, and chromosomal localization of human syntaxin 8 (STX8).
Cystic Fibrosis
Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas.
Cystic Fibrosis
Molecular determinants and role of an anion binding site in the external mouth of the CFTR chloride channel pore.
Cystic Fibrosis
Molecular determinants of anion selectivity in the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Cystic Fibrosis
Molecular determinants of Au(CN)(2)(-) binding and permeability within the cystic fibrosis transmembrane conductance regulator Cl(-) channel pore.
Cystic Fibrosis
Molecular Diagnosis and Genetic Counseling of Cystic Fibrosis and Related Disorders: New Challenges.
Cystic Fibrosis
Molecular diagnosis of congenital bilateral absence of the vas deferens: analyses of the CFTR gene in 64 French patients.
Cystic Fibrosis
Molecular diagnosis of cystic fibrosis in Indian patients--a preliminary report.
Cystic Fibrosis
Molecular Diagnostics of Cystic Fibrosis in Serbia: Our Approach to Meet the Diagnostic Challenges.
Cystic Fibrosis
Molecular dissection of the butyrate action revealed the involvement of mitogen-activated protein kinase in cystic fibrosis transmembrane conductance regulator biogenesis.
Cystic Fibrosis
Molecular Docking and QSAR Studies as Computational Tools Exploring the Rescue Ability of F508del CFTR Correctors.
Cystic Fibrosis
Molecular domains in epithelial salt cellNaCl of crustacean salt gland (Artemia).
Cystic Fibrosis
Molecular Dynamics Flexible Fitting Simulations Identify New Models of the Closed State of the Cystic Fibrosis Transmembrane Conductance Regulator Protein.
Cystic Fibrosis
Molecular dynamics simulation study on the structural instability of the most common cystic fibrosis-associated mutant ?F508-CFTR.
Cystic Fibrosis
Molecular Genetics of Cystic Fibrosis Transmembrane Conductance Regulator: Genotype and Phenotype.
Cystic Fibrosis
Molecular mechanism mediating enteric bacterial translocation after severe burn: the role of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Molecular mechanism of arachidonic acid inhibition of the CFTR chloride channel.
Cystic Fibrosis
Molecular mechanisms of calcium-sensing receptor-mediated calcium signaling in the modulation of epithelial ion transport and bicarbonate secretion.
Cystic Fibrosis
Molecular mechanisms of Cl- transport in fishes: New insights and their evolutionary context.
Cystic Fibrosis
Molecular mechanisms of pancreatic stone formation in chronic pancreatitis.
Cystic Fibrosis
Molecular modeling of the heterodimer of human CFTR's nucleotide-binding domains using a protein-protein docking approach.
Cystic Fibrosis
Molecular modelling approaches for cystic fibrosis transmembrane conductance regulator studies.
Cystic Fibrosis
Molecular models of the open and closed states of the whole human CFTR protein.
Cystic Fibrosis
Molecular pathophysiology of cystic fibrosis based on the rescued knockout mouse model.
Cystic Fibrosis
Molecular proximity of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel assessed by fluorescence resonance energy transfer.
Cystic Fibrosis
Molecular receptive range variation among mouse odorant receptors for aliphatic carboxylic acids.
Cystic Fibrosis
Molecular screening of the CFTR gene in men with anomalies of the vas deferens: identification of three novel mutations.
Cystic Fibrosis
Molecular species compositions of lung and pancreas phospholipids in the cftr(tm1HGU/tm1HGU) cystic fibrosis mouse.
Cystic Fibrosis
Molecular study of (TG)m(T)n polymorphisms in Iranian males with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
Molecular temporal bone pathology: III. Genotyping of the deltaF508 deletion in the DNA of patients with cystic fibrosis.
Cystic Fibrosis
Molecular understanding of chronic pancreatitis: a perspective on the future.
Cystic Fibrosis
MONNA, a Potent and Selective Blocker for Transmembrane Protein with Unknown Function 16/Anoctamin-1.
Cystic Fibrosis
Monoclonal antibodies against MDR1 P-glycoprotein inhibit chloride conductance and label a 65-kDa protein in pancreatic zymogen granule membranes.
Cystic Fibrosis
Monoclonal antibody against conductive chloride transport in pig ileal apical membrane vesicles.
Cystic Fibrosis
Monocytes from cystic fibrosis patients are locked in an LPS tolerance state: down-regulation of TREM-1 as putative underlying mechanism.
Cystic Fibrosis
Monomeric CFTR in plasma membranes in live cells revealed by single molecule fluorescence imaging.
Cystic Fibrosis
Morbidity and mortality in carriers of the cystic fibrosis mutation CFTR Phe508del in the general population.
Cystic Fibrosis
Morphologic effects of epithelial ion channels on the mouse uterus: differences between raloxifene analog (LY117018) and estradiol treatments.
Cystic Fibrosis
Morphological and functional heterogeneity of the mouse intrahepatic biliary epithelium.
Cystic Fibrosis
Morphological changes in the vas deferens and expression of the cystic fibrosis transmembrane conductance regulator (CFTR) in control, deltaF508 and knock-out CFTR mice during postnatal life.
Cystic Fibrosis
Morphological, molecular, and functional heterogeneity of cholangiocytes from normal rat liver.
Cystic Fibrosis
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin.
Cystic Fibrosis
Motion--genetic testing is useful in the diagnosis of nonhereditary pancreatic conditions: arguments against the motion.
Cystic Fibrosis
Motion--genetic testing is useful in the diagnosis of nonhereditary pancreatic conditions: arguments for the motion.
Cystic Fibrosis
Mouse cystic fibrosis transmembrane conductance regulator forms cAMP-PKA-regulated apical chloride channels in cortical collecting duct.
Cystic Fibrosis
Mouse models of chronic lung infection with Pseudomonas aeruginosa: models for the study of cystic fibrosis.
Cystic Fibrosis
Mouse organoid culture is a suitable model to study esophageal ion transport mechanisms.
Cystic Fibrosis
mRNA transfection by a Xentry-protamine cell-penetrating peptide is enhanced by TLR antagonist E6446.
Cystic Fibrosis
MRP class of human ATP binding cassette (ABC) transporters: historical background and new research directions.
Cystic Fibrosis
MRP transporters as membrane machinery in the bradykinin-inducible export of ATP.
Cystic Fibrosis
Mu 2 binding directs the cystic fibrosis transmembrane conductance regulator to the clathrin-mediated endocytic pathway.
Cystic Fibrosis
mu-opioid receptor regulates CFTR coexpressed in Xenopus oocytes in a cAMP independent manner.
Cystic Fibrosis
MUC4 expression is regulated by cystic fibrosis transmembrane conductance regulator in pancreatic adenocarcinoma cells via transcriptional and post-translational mechanisms.
Cystic Fibrosis
Mucin glycosylation and sulphation in airway epithelial cells is not influenced by cystic fibrosis transmembrane conductance regulator expression.
Cystic Fibrosis
MUCLIN expression in the cystic fibrosis transmembrane conductance regulator knockout mouse.
Cystic Fibrosis
Mucociliary clearance in cystic fibrosis knockout mice infected with Pseudomonas aeruginosa.
Cystic Fibrosis
Mucus secretion by single tracheal submucosal glands from normal and cystic fibrosis transmembrane conductance regulator knockout mice.
Cystic Fibrosis
Multi-dimensional clinical phenotyping of a national cohort of adult cystic fibrosis patients.
Cystic Fibrosis
Multi-Ion mechanism for ion permeation and block in the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Multiallelic Synthetic Quality Control Material: Lessons Learned from the Cystic Fibrosis External Quality Assessment Scheme.
Cystic Fibrosis
Multicenter characterization and validation of the intron-8 poly(T) tract (IVS8-T) status in 25 Coriell cell repository cystic fibrosis reference cell lines for cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation assays.
Cystic Fibrosis
Multicenter Intestinal Current Measurements in Rectal Biopsies from CF and Non-CF Subjects to Monitor CFTR Function.
Cystic Fibrosis
Multidimensional clinical phenotyping of an adult cystic fibrosis patient population.
Cystic Fibrosis
Multidrug resistance protein structure of Trypanosoma evansi isolated from buffaloes in Ngawi District, Indonesia: A bioinformatics analysis.
Cystic Fibrosis
Multidrug resistance-associated proteins: Export pumps for conjugates with glutathione, glucuronate or sulfate.
Cystic Fibrosis
Multifaceted structures and mechanisms of ABC transport systems in health and disease.
Cystic Fibrosis
Multifactorial Genesis of Pancreatitis in Primary Hyperparathyroidism: Evidence for "Protective" (PRSS2) and "Destructive" (CTRC) Genetic Factors.
Cystic Fibrosis
Multifunctional transporter models: lessons from the transport of water, sugars, and ring compounds by GLUTs.
Cystic Fibrosis
Multiple endocytic signals in the C-terminal tail of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Multiple inhibitory effects of Au(CN)(2-) ions on cystic fibrosis transmembrane conductance regulator Cl(-) channel currents.
Cystic Fibrosis
Multiple intracellular pathways for regulation of chloride secretion in cultured pig tracheal submucosal gland cells.
Cystic Fibrosis
Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gating.
Cystic Fibrosis
Multiple mutations in highly conserved residues are found in mildly affected cystic fibrosis patients.
Cystic Fibrosis
Multiple proteolytic systems, including the proteasome, contribute to CFTR processing.
Cystic Fibrosis
Multiple transport functions of a red blood cell anion exchanger, tAE1: its role in cell volume regulation.
Cystic Fibrosis
Multiplex allele-specific fluorescent PCR for haplotyping the IVS8 (TG)m(T)n locus in the CFTR gene.
Cystic Fibrosis
Multiplex PCR of polymorphic markers flanking the CFTR gene; a general approach for preimplantation genetic diagnosis of cystic fibrosis.
Cystic Fibrosis
Multiplexed detection of DOCK8, PGM3 and STAT3 proteins for the diagnosis of Hyper-Immunoglobulin E syndrome using gold nanoparticles-based immunosensor array platform.
Cystic Fibrosis
Multiprobe Nuclear Imaging of the Cystic Fibrosis Lung as a Biomarker of Therapeutic Effect.
Cystic Fibrosis
Multiscale approach to link red blood cell dynamics, shear viscosity, and ATP release.
Cystic Fibrosis
Multitarget CFTR Modulators Endowed with Multiple Beneficial Side Effects for Cystic Fibrosis Patients: Toward a Simplified Therapeutic Approach .
Cystic Fibrosis
Mummichog gill and operculum exhibit functionally consistent claudin-10 paralog profiles and Claudin-10c hypersaline response.
Cystic Fibrosis
Murine colonic mucosa hyperproliferation. I. Elevated CFTR expression and enhanced cAMP-dependent Cl(-) secretion.
Cystic Fibrosis
Murine colonic mucosa hyperproliferation. II. PKC-beta activation and cPKC-mediated cellular CFTR overexpression.
Cystic Fibrosis
Murine mCLCA6 is an integral apical membrane protein of non-goblet cell enterocytes and co-localizes with the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Muscarinic regulation of the cardiac CFTR Cl- current by quaternary ammonium compounds.
Cystic Fibrosis
Mutant (delta F508) cystic fibrosis transmembrane conductance regulator Cl- channel is functional when retained in endoplasmic reticulum of mammalian cells.
Cystic Fibrosis
Mutant cycles at CFTR's non-canonical ATP-binding site support little interface separation during gating.
Cystic Fibrosis
Mutant cystic fibrosis transmembrane conductance regulator inhibits acidification and apoptosis in C127 cells: possible relevance to cystic fibrosis.
Cystic Fibrosis
Mutating the Conserved Q-loop Glutamine 1291 Selectively Disrupts Adenylate Kinase-dependent Channel Gating of the ATP-binding Cassette (ABC) Adenylate Kinase Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) and Reduces Channel Function in Primary Human Airway Epithelia.
Cystic Fibrosis
Mutation Analysis of Exons 10 and 17a of CFTR Gene in Patients with Cystic Fibrosis in Kermanshah Province, Western Iran.
Cystic Fibrosis
Mutation analysis of PRSS1, SPINK1 and CFTR gene in patients with alcoholic and idiopathic chronic pancreatitis: A single center study.
Cystic Fibrosis
Mutation Analysis of the CFTR Gene in 225 Children: Identification of Five Novel Severe and Seven Reported Severe Mutations.
Cystic Fibrosis
Mutation analysis of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, the cationic trypsinogen (PRSS1) gene, and the serine protease inhibitor, Kazal type 1 (SPINK1) gene in patients with alcoholic chronic pancreatitis.
Cystic Fibrosis
Mutation analysis of the cystic fibrosis transmembrane conductance regulator gene in Chinese congenital absence of vas deferens patients.
Cystic Fibrosis
Mutation analysis of the cystic fibrosis transmembrane regulator gene in Native American populations of the southwest.
Cystic Fibrosis
Mutation and new polymorphisms insight in introns 11 to 14a of CFTR gene of northern Iranian cystic fibrosis patients.
Cystic Fibrosis
Mutation frequency of cystic fibrosis transmembrane regulator is not increased in oligozoospermic male candidates for intracytoplasmic sperm injection.
Cystic Fibrosis
Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population.
Cystic Fibrosis
Mutation nomenclature in practice: Findings and recommendations from the cystic fibrosis external quality assessment scheme.
Cystic Fibrosis
Mutation of potential phosphorylation sites in the recombinant R domain of the cystic fibrosis transmembrane conductance regulator has significant effects on domain conformation.
Cystic Fibrosis
Mutation of the aromatic amino acid interacting with adenine moiety of ATP to a polar residue alters the properties of multidrug resistance protein 1.
Cystic Fibrosis
Mutation of Walker-A lysine 464 in cystic fibrosis transmembrane conductance regulator reveals functional interaction between its nucleotide-binding domains.
Cystic Fibrosis
Mutation Studies in the CFTR Gene in Asian Indian Subjects with Congenital Bilateral Absence of Vas Deferens: Report of Two Novel Mutations and Four Novel Variants.
Cystic Fibrosis
Mutation-induced blocker permeability and multiion block of the CFTR chloride channel pore.
Cystic Fibrosis
Mutation-specific potency and efficacy of cystic fibrosis transmembrane conductance regulator chloride channel potentiators.
Cystic Fibrosis
Mutational analysis of CFTR in the Ecuadorian population using next-generation sequencing.
Cystic Fibrosis
Mutational analysis of the Saccharomyces cerevisiae ATP-binding cassette transporter protein Ycf1p.
Cystic Fibrosis
Mutational disruption of plasma membrane trafficking of Saccharomyces cerevisiae Yor1p, a homologue of mammalian multidrug resistance protein.
Cystic Fibrosis
Mutations and sequence variations detected in the cystic fibrosis transmembrane conductance regulator (CFTR) gene: a report from the Cystic Fibrosis Genetic Analysis Consortium.
Cystic Fibrosis
Mutations at the signature sequence of CFTR create a Cd(2+)-gated chloride channel.
Cystic Fibrosis
Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties.
Cystic Fibrosis
Mutations in CFTR gene and clinical correlation in Argentine patients with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
Mutations in the amiloride-sensitive epithelial sodium channel in patients with cystic fibrosis-like disease.
Cystic Fibrosis
Mutations in the amino terminus of the cystic fibrosis transmembrane conductance regulator enhance endocytosis.
Cystic Fibrosis
Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens.
Cystic Fibrosis
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) in Chinese patients with congenital bilateral absence of vas deferens.
Cystic Fibrosis
Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials.
Cystic Fibrosis
Mutations in the cystic fibrosis transmembrane regulator gene in patients with tropical calcific pancreatitis.
Cystic Fibrosis
Mutations in the DNAH11 (axonemal heavy chain dynein type 11) gene cause one form of situs inversus totalis and most likely primary ciliary dyskinesia.
Cystic Fibrosis
Mutations in the nucleotide binding domain 1 signature motif region rescue processing and functional defects of cystic fibrosis transmembrane conductance regulator delta f508.
Cystic Fibrosis
Mutations in the putative pore-forming domain of CFTR do not change anion selectivity of the cAMP activated Cl- conductance.
Cystic Fibrosis
Mutations of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene Cause a Monocyte-selective Adhesion Deficiency.
Cystic Fibrosis
Mutations of the CFTR gene in Turkish patients with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
Mutations of the cystic fibrosis gene and intermediate sweat chloride levels in children.
Cystic Fibrosis
Mutations of the cystic fibrosis gene in patients with chronic pancreatitis.
Cystic Fibrosis
Mutations of the cystic fibrosis gene, but not cationic trypsinogen gene, are associated with recurrent or chronic idiopathic pancreatitis.
Cystic Fibrosis
Mutations of the cystic fibrosis transmembrane conductance regulator gene in males with congenital bilateral absence of the vas deferens: Reproductive implications and genetic counseling (Review).
Cystic Fibrosis
Mutations that change the position of the putative gamma-phosphate linker in the nucleotide binding domains of CFTR alter channel gating.
Cystic Fibrosis
Mutual Enhancement of Virulence by Enterotoxigenic and Enteropathogenic Escherichia coli.
Cystic Fibrosis
Myosin Vb is required for trafficking of the cystic fibrosis transmembrane conductance regulator in Rab11a-specific apical recycling endosomes in polarized human airway epithelial cells.
Cystic Fibrosis
Myosin VI regulates endocytosis of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
N-glycans are direct determinants of CFTR folding and stability in secretory and endocytic membrane traffic.
Cystic Fibrosis
N-terminal CFTR missense variants severely affect the behavior of the CFTR chloride channel.
Cystic Fibrosis
N-terminal myristoylation is required for membrane localization of cGMP-dependent protein kinase type II.
Cystic Fibrosis
N1303K (c.3909C>G) mutation and splicing: implication of its c.[744-33GATT(6); 869+11C>T] complex allele in CFTR exon 7 aberrant splicing.
Cystic Fibrosis
Na+ and Cl- conductances in airway epithelial cells: increased Na+ conductance in cystic fibrosis.
Cystic Fibrosis
Na+/H+ exchanger regulatory factor 1 overexpression-dependent increase of cytoskeleton organization is fundamental in the rescue of F508del cystic fibrosis transmembrane conductance regulator in human airway CFBE41o- cells.
Cystic Fibrosis
Na+/H+ exchanger regulatory factor isoform 1 overexpression modulates cystic fibrosis transmembrane conductance regulator (CFTR) expression and activity in human airway 16HBE14o- cells and rescues DeltaF508 CFTR functional expression in cystic fibrosis cells.
Cystic Fibrosis
Na+/K+/2Cl- cotransporter and CFTR gill expression after seawater transfer in smolts (0+) of different Atlantic salmon (Salmo salar) families
Cystic Fibrosis
NaCl and fluid secretion by the intestine of the teleost Fundulus heteroclitus: involvement of CFTR.
Cystic Fibrosis
Nanobody-based binding assay for the discovery of potent inhibitors of CFTR inhibitory factor (Cif).
Cystic Fibrosis
Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating.
Cystic Fibrosis
Nanomolar CFTR inhibition by pore-occluding divalent polyethylene glycol-malonic acid hydrazides.
Cystic Fibrosis
Nanomolar Potency Pyrimido-pyrrolo-quinoxalinedione CFTR Inhibitor Reduces Cyst Size in a Polycystic Kidney Disease Model.
Cystic Fibrosis
Nanomolar-potency 'co-potentiator' therapy for cystic fibrosis caused by a defined subset of minimal function CFTR mutants.
Cystic Fibrosis
Nanomolar-Potency Aminophenyl-1,3,5-triazine Activators of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Chloride Channel for Prosecretory Therapy of Dry Eye Diseases.
Cystic Fibrosis
Nanoparticles that deliver triplex-forming peptide nucleic acid molecules correct F508del CFTR in airway epithelium.
Cystic Fibrosis
Naringenin Regulates CFTR Activation and Expression in Airway Epithelial Cells.
Cystic Fibrosis
Nasal application of the cationic liposome DC-Chol:DOPE does not alter ion transport, lung function or bacterial growth.
Cystic Fibrosis
Nasal potential difference in non-classic cystic fibrosis-long term follow up.
Cystic Fibrosis
Nasal potential difference measurements in patients with atypical cystic fibrosis.
Cystic Fibrosis
Nasal potential measurements on the nasal floor and under the inferior turbinate: Does it matter?
Cystic Fibrosis
NDPK-A (but not NDPK-B) and AMPK alpha1 (but not AMPK alpha2) bind the cystic fibrosis transmembrane conductance regulator in epithelial cell membranes.
Cystic Fibrosis
Nebulisation of receptor-targeted nanocomplexes for gene delivery to the airway epithelium.
Cystic Fibrosis
Negative chronotropic and inotropic effects of lubiprostone on iPS cell-derived cardiomyocytes via activation of CFTR.
Cystic Fibrosis
Negative regulation of CFTR activity by extracellular ATP involves P2Y2 receptors in CFTR-expressing CHO cells.
Cystic Fibrosis
Negative sweat test in hypertrypsinaemic infants with cystic fibrosis carrying rare CFTR mutations.
Cystic Fibrosis
Neuraminidase inhibitors for the treatment of influenza infection in people with cystic fibrosis.
Cystic Fibrosis
Neuropeptide regulation of secretion and inflammation in human airway gland serous cells.
Cystic Fibrosis
Neutrophil elastase degrades cystic fibrosis transmembrane conductance regulator via calpains and disables channel function in vitro and in vivo.
Cystic Fibrosis
Neutrophil extracellular trap (NET)-mediated killing of Pseudomonas aeruginosa: evidence of acquired resistance within the CF airway, independent of CFTR.
Cystic Fibrosis
New generation ENaC inhibitors detach cystic fibrosis airway mucus bundles via sodium/hydrogen exchanger inhibition.
Cystic Fibrosis
New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation.
Cystic Fibrosis
New Insights into the Binding Features of F508del CFTR Potentiators: A Molecular Docking, Pharmacophore Mapping and QSAR Analysis Approach.
Cystic Fibrosis
New insights into the mechanisms underlying hydrosalpinx fluid formation and its adverse effect on IVF outcome.
Cystic Fibrosis
New insights into the pathogenesis of cystic fibrosis: pivotal role of glutathione system dysfunction and implications for therapy.
Cystic Fibrosis
New Model of Cystic Fibrosis Transmembrane Conductance Regulator Proposes Active Channel-like Conformation.
Cystic Fibrosis
New models of the tracheal airway define the glandular contribution to airway surface fluid and electrolyte composition.
Cystic Fibrosis
New pancreas cancers cell lines that represent distinct stages of ductal differentiation.
Cystic Fibrosis
New Therapeutic Approaches to Modulate and Correct Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
Newborn cystic fibrosis screening in southeastern Mexico: Birth prevalence and novel CFTR gene variants.
Cystic Fibrosis
Newborn screening for cystic fibrosis in Wisconsin: comparison of biochemical and molecular methods.
Cystic Fibrosis
Next-Generation Sequencing for Molecular Diagnosis of Cystic Fibrosis in a Brazilian Cohort.
Cystic Fibrosis
NF kappaB expression increases and CFTR and MUC1 expression decreases in the endometrium of infertile patients with hydrosalpinx: a comparative study.
Cystic Fibrosis
NF-kappa B mediates up-regulation of CFTR gene expression in Calu-3 cells by interleukin-1beta.
Cystic Fibrosis
NF-kappaB activation is involved in regulation of cystic fibrosis transmembrane conductance regulator (CFTR) by interleukin-1beta.
Cystic Fibrosis
Nickel inhibits ?-1 adrenoceptor mediated activation of cardiac CFTR chloride channels.
Cystic Fibrosis
Nicotine Induces Progressive Properties of Lung Adenocarcinoma A549 Cells by Inhibiting Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Expression and Plasma Membrane Localization.
Cystic Fibrosis
Nine cystic fibrosis patients homozygous for the CFTR nonsense mutation R1162X have mild or moderate lung disease.
Cystic Fibrosis
Nine mutations in the cystic fibrosis (CF) gene account for 80% of the CF chromosomes in French patients.
Cystic Fibrosis
Nitric oxide has no beneficial effects on ion transport defects in cystic fibrosis human nasal epithelium.
Cystic Fibrosis
Nitric oxide inhibits heterologous CFTR expression in polarized epithelial cells.
Cystic Fibrosis
Nitric oxide synthase 1 as a potential modifier gene of decline in lung function in patients with cystic fibrosis.
Cystic Fibrosis
NLR family pyrin domain containing 3 (NLRP3) and caspase 1 (CASP1) modulation by intracellular Cl- concentration.
Cystic Fibrosis
NMR evidence for differential phosphorylation-dependent interactions in WT and DeltaF508 CFTR.
Cystic Fibrosis
NMR measurements of the diffusional permeability of water in cultured colonic epithelial cancer cells.
Cystic Fibrosis
NMR structure of a type IVb pilin from Salmonella typhi and its assembly into pilus.
Cystic Fibrosis
No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations.
Cystic Fibrosis
No evidence for direct activation of the cystic fibrosis transmembrane conductance regulator by 8-cyclopentyl-1,3-dipropylxanthine.
Cystic Fibrosis
No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis.
Cystic Fibrosis
NO inhibits signal transduction pathway for ATP release from erythrocytes via its action on heterotrimeric G protein Gi.
Cystic Fibrosis
Nobiletin Stimulates Chloride Secretion in Human Bronchial Epithelia via a cAMP/PKA-Dependent Pathway.
Cystic Fibrosis
Non-conventional trafficking of the cystic fibrosis transmembrane conductance regulator through the early secretory pathway.
Cystic Fibrosis
Non-Genomic Effects of Fluticasone Propionate and Budesonide on Human Airway Anion Secretion.
Cystic Fibrosis
Non-isocratic capillary electrophoresis for detection of DNA point mutations.
Cystic Fibrosis
Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation.
Cystic Fibrosis
Non-native interhelical hydrogen bonds in the cystic fibrosis transmembrane conductance regulator domain modulated by polar mutations.
Cystic Fibrosis
Non-pore lining amino acid side chains influence anion selectivity of the human CFTR Cl- channel expressed in mammalian cell lines.
Cystic Fibrosis
Non-radioactive detection of the most common mutations in the cystic fibrosis transmembrane conductance regulator gene by multiplex allele-specific polymerase chain reaction.
Cystic Fibrosis
Non-specific activation of the epithelial sodium channel by the CFTR chloride channel.
Cystic Fibrosis
Non-specificity of chloride channel blockers in rat cerebral arteries: block of the L-type calcium channel.
Cystic Fibrosis
Non-viral mediated gene therapy in human cystic fibrosis airway epithelial cells recovers chloride channel functionality.
Cystic Fibrosis
Nonintegral stoichiometry in CFTR gating revealed by a pore-lining mutation.
Cystic Fibrosis
Nonisotopic detection of mutations using a modified single-strand conformation polymorphism analysis.
Cystic Fibrosis
Nonradiometric ELISA-based quantitation and validation of polymerase chain reaction-amplified DNA, including detection of point mutations, without allele-specific amplification, or ligation.
Cystic Fibrosis
Nonselective cation and Cl channels in luminal membrane of the marginal cell.
Cystic Fibrosis
Nonsense mutation R1162X of the cystic fibrosis transmembrane conductance regulator gene does not reduce messenger RNA expression in nasal epithelial tissue.
Cystic Fibrosis
Nonsteroidal anti-inflammatory drugs upregulate function of wild-type and mutant CFTR.
Cystic Fibrosis
Nonstructural Protein NP1 of Human Bocavirus 1 Plays a Critical Role in the Expression of Viral Capsid Proteins.
Cystic Fibrosis
Nontruncating SCN1A mutations associated with severe myoclonic epilepsy of infancy impair cell surface expression.
Cystic Fibrosis
Nonvisualization of the Fetal Gallbladder: Can Levels of Gamma-Glutamyl Transpeptidase in Amniotic Fluid Predict Fetal Prognosis?
Cystic Fibrosis
Normal CFTR Inhibits Epidermal Growth Factor Receptor-Dependent Pro-Inflammatory Chemokine Production in Human Airway Epithelial Cells.
Cystic Fibrosis
Normal function of the cystic fibrosis conductance regulator protein can be associated with homozygous (Delta)F508 mutation.
Cystic Fibrosis
Normal mucus formation requires cAMP-dependent HCO3- secretion and Ca2+-mediated mucin exocytosis.
Cystic Fibrosis
Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelial cells.
Cystic Fibrosis
Novel action of the chalcone isoliquiritigenin as a cystic fibrosis transmembrane conductance regulator (CFTR) inhibitor: potential therapy for cholera and polycystic kidney disease.
Cystic Fibrosis
Novel Action of the Chalcone Isoliquiritigenin as a Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Inhibitor: Potential Therapy for Cholera and Polycystic Kidney Disease.
Cystic Fibrosis
Novel amino-carbonitrile-pyrazole identified in a small molecule screen activates wild-type and ?F508 cystic fibrosis transmembrane conductance regulator in the absence of a cAMP agonist.
Cystic Fibrosis
Novel and recurrent rearrangements in the CFTR gene: clinical and laboratory implications for cystic fibrosis screening.
Cystic Fibrosis
Novel ANO1 Inhibitor from Mallotus apelta Extract Exerts Anticancer Activity through Downregulation of ANO1.
Cystic Fibrosis
Novel association of HLA-haplotypes with primary sclerosing cholangitis (PSC) in a southern European population.
Cystic Fibrosis
Novel cause of hereditary obstructive azoospermia: a T2 allele in the CFTR gene.
Cystic Fibrosis
Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquinolizinium lead compounds.
Cystic Fibrosis
Novel cftr gene sequence variation in serbian patient with idiopathic disseminated bronchiectasis.
Cystic Fibrosis
Novel CFTR Variants Identified during the First 3 Years of Cystic Fibrosis Newborn Screening in California.
Cystic Fibrosis
Novel Correctors and Potentiators Enhance Functional Rescue of CFTR Nonsense Mutation Translational Readthrough.
Cystic Fibrosis
Novel cystic fibrosis mutation associated with mild disease in Cypriot patients.
Cystic Fibrosis
Novel de novo large deletion in cystic fibrosis transmembrane conductance regulator gene results in a severe cystic fibrosis phenotype.
Cystic Fibrosis
Novel frameshift variant of the CFTR gene: S511Lfs*2 from phenotype to molecular predictions.
Cystic Fibrosis
Novel GUCY2C variant causing familial diarrhea in a Mennonite kindred and a potential therapeutic approach.
Cystic Fibrosis
Novel Hits in the Correction of ?F508-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Protein: Synthesis, Pharmacological, and ADME Evaluation of Tetrahydropyrido[4,3-d]pyrimidines for the Potential Treatment of Cystic Fibrosis.
Cystic Fibrosis
Novel Insight Into the Role of CFTR in Lacrimal Gland Duct Function in Mice.
Cystic Fibrosis
Novel mutation c.1210-3C > G in cis with a poly-T tract of 5T affects CFTR mRNA splicing in a Chinese patient with cystic fibrosis.
Cystic Fibrosis
Novel mutations and deletions in cystic fibrosis in a tertiary cystic fibrosis center in Istanbul.
Cystic Fibrosis
Novel mutations and polymorphisms in the CFTR gene associated with three subtypes of congenital absence of vas deferens.
Cystic Fibrosis
Novel picolinamide-based cystic fibrosis transmembrane regulator modulators: evaluation of WO2013038373, WO2013038376, WO2013038381, WO2013038386 and WO2013038390.
Cystic Fibrosis
Novel pore-lining residues in CFTR that govern permeation and open-channel block.
Cystic Fibrosis
Novel regulation of cystic fibrosis transmembrane conductance regulator (CFTR) channel gating by external chloride.
Cystic Fibrosis
Novel residues lining the CFTR chloride channel pore identified by functional modification of introduced cysteines.
Cystic Fibrosis
Novel role for cystic fibrosis transmembrane conductance regulator in alveolar fluid clearance in lipopolysaccharide-induced acute lung injury in mice.
Cystic Fibrosis
Novel role for pendrin in orchestrating bicarbonate secretion in cystic fibrosis transmembrane conductance regulator (CFTR)-expressing airway serous cells.
Cystic Fibrosis
Novel role of cystic fibrosis transmembrane conductance regulator in maintaining adult mouse olfactory neuronal homeostasis.
Cystic Fibrosis
Novel short chain fatty acids restore chloride secretion in cystic fibrosis.
Cystic Fibrosis
Novel SIN-1 reactive intermediates modulate chloride secretion across murine airway cells.
Cystic Fibrosis
Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping.
Cystic Fibrosis
Nucleosome mapping across the CFTR locus identifies novel regulatory factors.
Cystic Fibrosis
Nucleotide occlusion in the human cystic fibrosis transmembrane conductance regulator. Different patterns in the two nucleotide binding domains.
Cystic Fibrosis
Nucleotide release provides a mechanism for airway surface liquid homeostasis.
Cystic Fibrosis
Nucleotide transport through the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Nucleotide-binding domain 1 of cystic fibrosis transmembrane conductance regulator production of a suitable protein for structural studies.
Cystic Fibrosis
Nucleotide-binding Domains of Cystic Fibrosis Transmembrane Conductance Regulator, an ABC Transporter, Catalyze Adenylate Kinase Activity but Not ATP Hydrolysis.
Cystic Fibrosis
Nucleotide-binding domains of human cystic fibrosis transmembrane conductance regulator: detailed sequence analysis and three-dimensional modeling of the heterodimer.
Cystic Fibrosis
Nutritional effects on host response to lung infections with mucoid Pseudomonas aeruginosa in mice.
Cystic Fibrosis
Nutritional status and eating disorders: neglected risks factor for nontuberculous mycobacterial lung disease?
Cystic Fibrosis
Obliterative Portal Venopathy Without Cirrhosis Is Prevalent in Pediatric Cystic Fibrosis Liver Disease With Portal Hypertension.
Cystic Fibrosis
Ocular Surface Potential Difference Measured in Human Subjects to Study Ocular Surface Ion Transport.
Cystic Fibrosis
Omission of exon 12 in cystic fibrosis transmembrane conductance regulator (CFTR) gene transcripts.
Cystic Fibrosis
On the interactions between nucleotide binding domains and membrane spanning domains in cystic fibrosis transmembrane regulator: A molecular dynamic study.
Cystic Fibrosis
On the mechanism of genistein-induced activation of protein kinase A-dependent Cl- conductance in cardiac myocytes.
Cystic Fibrosis
On the origin of asymmetric interactions between permeant anions and the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Cystic Fibrosis
On the relationship between anion binding and chloride conductance in the CFTR anion channel.
Cystic Fibrosis
One-step affinity isolation of recombinant protein using the baculovirus/insect cell expression system.
Cystic Fibrosis
Ontogeny of CLCN3 chloride channel gene expression in human pulmonary epithelium.
Cystic Fibrosis
Opening up of plasmalemma type-1 VDAC to form apoptotic "find me signal" pathways is essential in early apoptosis - Evidence from the pathogenesis of cystic fibrosis resulting from failure of apoptotic cell clearance followed by sterile inflammation.
Cystic Fibrosis
Opiates inhibit ion conductances elicited by cell swelling and cAMP in cultured cells.
Cystic Fibrosis
Opportunistic infections in lung disease: Pseudomonas infections in cystic fibrosis.
Cystic Fibrosis
Optical imaging of Ca2+-evoked fluid secretion by murine nasal submucosal gland serous acinar cells.
Cystic Fibrosis
Optical imaging of Cl- permeabilities in normal and CFTR-expressing mouse L cells.
Cystic Fibrosis
Optimal Complement-Mediated Phagocytosis of Pseudomonas aeruginosa by Monocytes Is Cystic Fibrosis Transmembrane Conductance Regulator-Dependent.
Cystic Fibrosis
Optimal correction of distinct CFTR folding mutants in rectal cystic fibrosis organoids.
Cystic Fibrosis
Optimisation of real-time quantitative RT-PCR for the evaluation of non-viral mediated gene transfer to the airways.
Cystic Fibrosis
Optimization of a Yellow fluorescent protein-based iodide influx high-throughput screening assay for cystic fibrosis transmembrane conductance regulator (CFTR) modulators.
Cystic Fibrosis
Optimization of cationic liposome-mediated gene transfer to human bronchial epithelial cells expressing wild-type or abnormal cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
Optimization of rAAV-mediated Expression for Large Transgenes Using a Synthetic Promoter and Tandem Array Enhancers.
Cystic Fibrosis
Optimization of the degenerated interfacial ATP binding site improves the function of diseases related mutant cystic fibrosis transmembrane conductance regulator (CFTR) channels.
Cystic Fibrosis
Optimized detection of DNA point mutations by double gradient denaturing gradient gel electrophoresis.
Cystic Fibrosis
Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation.
Cystic Fibrosis
Organ-specific over-sulfation of glycosaminoglycans and altered extracellular matrix in a mouse model of cystic fibrosis.
Cystic Fibrosis
Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Oridonin: A small molecule inhibitor of cystic fibrosis transmembrane conductance regulator (CFTR) isolated from traditional Chinese medicine.
Cystic Fibrosis
Origin and evolution of the cystic fibrosis transmembrane regulator protein R domain.
Cystic Fibrosis
ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling to the Cystic Fibrosis Transmembrane Conductance Regulator Channel.
Cystic Fibrosis
Orphan missense mutations in the cystic fibrosis transmembrane conductance regulator a three-step biological approach to establishing a correlation between genotype and phenotype.
Cystic Fibrosis
Osmoregulation in the Plotosidae Catfish: Role of the Salt Secreting Dendritic Organ.
Cystic Fibrosis
Osteoblast CFTR inactivation reduces differentiation and osteoprotegerin expression in a mouse model of cystic fibrosis-related bone disease.
Cystic Fibrosis
Osteoclastogenesis and sphingosine-1-phosphate secretion from human osteoclast precursor monocytes are modulated by the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
OTX2 regulates CFTR expression during endoderm differentiation and occupies 3' cis-regulatory elements.
Cystic Fibrosis
Ouabain Mimics Low Temperature Rescue of F508del-CFTR in Cystic Fibrosis Epithelial Cells.
Cystic Fibrosis
Ouabain Regulates CFTR-Mediated Anion Secretion and Na,K-ATPase Transport in ADPKD Cells.
Cystic Fibrosis
Outcome of intracytoplasmic sperm injection for a couple in which the man is carrier of CFTR p.[R74W;V201M;D1270N] and p.P841R mutations and his spouse a heterozygous carrier of p.F508del mutation of the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Overcoming Immunological Challenges to Helper-Dependent Adenoviral Vector-Mediated Long-Term CFTR Expression in Mouse Airways.
Cystic Fibrosis
Overexpression of cystic fibrosis transmembrane conductance regulator (CFTR) is associated with human cervical cancer malignancy, progression and prognosis.
Cystic Fibrosis
Overexpression of R domain eliminates cAMP-stimulated Cl- secretion in 9/HTEo- cells in culture.
Cystic Fibrosis
Overexpression of the cystic fibrosis transmembrane conductance regulator in NIH 3T3 cells lowers membrane potential and intracellular pH and confers a multidrug resistance phenotype.
Cystic Fibrosis
Overexpression, purification, and function of first nucleotide-binding fold of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Overproduction of the CFTR R domain leads to increased levels of asialoGM1 and increased Pseudomonas aeruginosa binding by epithelial cells.
Cystic Fibrosis
Ovine male genital duct epithelial cells differentiate in vitro and express functional CFTR and ENaC.
Cystic Fibrosis
Oxidant and antioxidant modulation of chloride channels expressed in human retinal pigment epithelium.
Cystic Fibrosis
Oxidant stress stimulates anion secretion from the human airway epithelial cell line Calu-3: implications for cystic fibrosis lung disease.
Cystic Fibrosis
Oxidative stress causes IL8 promoter hyperacetylation in cystic fibrosis airway cell models.
Cystic Fibrosis
Oxidative stress regulates CFTR gene expression in human airway epithelial cells through a distal antioxidant response element.
Cystic Fibrosis
Oxygen-glucose deprivation induces ATP release via maxi-anion channels in astrocytes.
Cystic Fibrosis
Ozone stress down-regulates the expression of cystic fibrosis transmembrane conductance regulator in human bronchial epithelial cells.
Cystic Fibrosis
P-glycoprotein expression increases ATP release in respiratory cystic fibrosis cells.
Cystic Fibrosis
p.F508del in a heterogeneous cystic fibrosis population from Minas Gerais, Brazil.
Cystic Fibrosis
p.Ser1235Arg should no longer be considered as a cystic fibrosis mutation: results from a large collaborative study.
Cystic Fibrosis
P2Y purinergic receptor regulation of CFTR chloride channels in mouse cardiac myocytes.
Cystic Fibrosis
P2Y6 receptor mediates colonic NaCl secretion via differential activation of cAMP-mediated transport.
Cystic Fibrosis
Pancreatic and Islet Remodeling in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Knockout Ferrets.
Cystic Fibrosis
Pancreatic insufficiency and pulmonary disease in German and Slavic cystic fibrosis patients with the R347P mutation.
Cystic Fibrosis
Pancreatic intraepithelial neoplasia is associated with chronic pancreatitis due to serine protease inhibitor kazal type 1 and cystic fibrosis transmembrane conductance regulator mutations.
Cystic Fibrosis
Pancreatitis risk in primary hyperparathyroidism: relation to mutations in the SPINK1 trypsin inhibitor (N34S) and the cystic fibrosis gene.
Cystic Fibrosis
Pancreatitis severity in mice with impaired CFTR function but pancreatic sufficiency is mediated via ductal and inflammatory cells-Not acinar cells.
Cystic Fibrosis
Paracellular bicarbonate flux across human cystic fibrosis airway epithelia tempers changes in airway surface liquid pH.
Cystic Fibrosis
Paracellular transport through healthy and cystic fibrosis bronchial epithelial cell lines--do we have a proper model?
Cystic Fibrosis
Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells.
Cystic Fibrosis
Parallel single nucleotide polymorphism genotyping by surface invasive cleavage with universal detection.
Cystic Fibrosis
Paranasal sinus size is decreased in CFTR heterozygotes with chronic rhinosinusitis.
Cystic Fibrosis
Parasympathetic innervation regulates tubulogenesis in the developing salivary gland.
Cystic Fibrosis
Parathyroid hormone increases CFTR expression and function in Caco-2 intestinal epithelial cells.
Cystic Fibrosis
Partial correction of endogenous DeltaF508 CFTR in human cystic fibrosis airway epithelia by spliceosome-mediated RNA trans-splicing.
Cystic Fibrosis
Partial purification of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Partial purification of the pig kidney cystic fibrosis transmembrane regulator protein.
Cystic Fibrosis
Participation of cAMP in a signal-transduction pathway relating erythrocyte deformation to ATP release.
Cystic Fibrosis
Participation of the Cl-/HCO(3)- exchangers SLC26A3 and SLC26A6, the Cl- channel CFTR, and the regulatory factor SLC9A3R1 in mouse sperm capacitation.
Cystic Fibrosis
Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Patch clamp on the luminal membrane of exocrine gland acini from frog skin (Rana esculenta) reveals the presence of cystic fibrosis transmembrane conductance regulator-like Cl- channels activated by cyclic AMP.
Cystic Fibrosis
Patch-clamp studies of cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases.
Cystic Fibrosis
Pathogen acquisition in patients with cystic fibrosis receiving ivacaftor or lumacaftor/ivacaftor.
Cystic Fibrosis
Pathogen and autoantigen homologous regions within the cystic fibrosis transmembrane conductance regulator (CFTR) protein suggest an autoimmune treatable component of cystic fibrosis.
Cystic Fibrosis
Pathogenic role of ADGRG2 in CBAVD patients replicated in Chinese population.
Cystic Fibrosis
Pathology of gastrointestinal organs in a porcine model of cystic fibrosis.
Cystic Fibrosis
Pathophysiologic consequences following inhibition of a CFTR-dependent developmental cascade in the lung.
Cystic Fibrosis
Pathophysiological mechanisms of diarrhea caused by the Vibrio cholerae O1 El Tor variant: an in vivo study in mice.
Cystic Fibrosis
Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota.
Cystic Fibrosis
Pathophysiology of Hyperechogenic Bowel in Congenitally Human Cytomegalovirus Infected Fetuses.
Cystic Fibrosis
Pathway prediction by bioinformatic analysis of the untranslated regions of the CFTR mRNA.
Cystic Fibrosis
Pathways in the Pathophysiology of Coronavirus 19 Lung Disease Accessible to Prevention and Treatment.
Cystic Fibrosis
Patient and family issues regarding genetic testing for cystic fibrosis: a review of prenatal carrier testing and newborn screening.
Cystic Fibrosis
Patient personalized translational tools in cystic fibrosis to transform data from bench to bed-side and back.
Cystic Fibrosis
Patient perspectives following initiation of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis and advanced lung disease.
Cystic Fibrosis
Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey.
Cystic Fibrosis
Patients with mutations in Gsalpha have reduced activation of a downstream target in epithelial tissues due to haploinsufficiency.
Cystic Fibrosis
Paucity of CFTR current but modest CFTR immunoreactivity in non-diseased human ventricle.
Cystic Fibrosis
PCR bias toward the wild-type k-ras and p53 sequences: implications for PCR detection of mutations and cancer diagnosis.
Cystic Fibrosis
PDE5 inhibitors for cystic fibrosis: can they also enhance chloride transport? Evaluation of: Lubamba B, Lecourt H, Lebacq J, et al. Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis. Am J Respir Crit Care Med 2008;177(5):506-15.
Cystic Fibrosis
PDZ domain interaction controls the endocytic recycling of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
PDZ Structure and Implication in Selective Drug Design against Cystic Fibrosis.
Cystic Fibrosis
PDZ-binding motifs are unable to ensure correct polarized protein distribution in the absence of additional localization signals.
Cystic Fibrosis
Pediatric population with cystic fibrosis in the centre of Portugal: Candidates for new therapies.
Cystic Fibrosis
Pendrin stimulates a chloride absorption pathway to increase CFTR-mediated chloride secretion from Cystic Fibrosis airway epithelia.
Cystic Fibrosis
Peptide binding consensus of the NHE-RF-PDZ1 domain matches the C-terminal sequence of cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
Peptide microarray analysis of substrate specificity of the transmembrane Ser/Thr kinase KPI-2 reveals reactivity with cystic fibrosis transmembrane conductance regulator and phosphorylase.
Cystic Fibrosis
Peptide surface display and secretion using two LPXTG-containing surface proteins from Lactobacillus fermentum BR11.
Cystic Fibrosis
Perinuclear positioning of the inactive human cystic fibrosis gene depends on CTCF, A-type lamins and an active histone deacetylase.
Cystic Fibrosis
Peripheral localization of the epithelial sodium channel in the apical membrane of bronchial epithelial cells.
Cystic Fibrosis
Peripheral Protein Quality Control as a Novel Drug Target for CFTR Stabilizer.
Cystic Fibrosis
Peripheral protein quality control removes unfolded CFTR from the plasma membrane.
Cystic Fibrosis
Permeability of wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride channels to polyatomic anions.
Cystic Fibrosis
Permissiveness of human biliary epithelial cells to infection by hepatitis C virus.
Cystic Fibrosis
Persistent interactions between the two transmembrane clusters dictate the targeting and functional assembly of adenylyl cyclase.
Cystic Fibrosis
Persistent osteopenia in adult cystic fibrosis transmembrane conductance regulator-deficient mice.
Cystic Fibrosis
Personalized medicine for cystic fibrosis: establishing human model systems.
Cystic Fibrosis
Perspectives on mucus properties and formation--lessons from the biochemical world.
Cystic Fibrosis
Perturbation of the pore of the cystic fibrosis transmembrane conductance regulator (CFTR) inhibits its atpase activity.
Cystic Fibrosis
PGE2 Regulation of CFTR Activity and Air Surface Liquid Volume Requires Gap Junctional Communication.
Cystic Fibrosis
Pharmaceuticals targeting nonsense mutations in genetic diseases: progress in development.
Cystic Fibrosis
Pharmacogenomics of the cystic fibrosis transmembrane conductance regulator (CFTR) and the cystic fibrosis drug CPX using genome microarray analysis.
Cystic Fibrosis
Pharmacokinetic and Drug-Drug Interaction Profiles of the Combination of Tezacaftor/Ivacaftor.
Cystic Fibrosis
Pharmacokinetics and safety of cavosonstat (N91115) in healthy and cystic fibrosis adults homozygous for F508DEL-CFTR.
Cystic Fibrosis
Pharmacologic modulation of salt and water in the airway epithelium in cystic fibrosis.
Cystic Fibrosis
Pharmacologic therapy for stop mutations: how much CFTR activity is enough?
Cystic Fibrosis
Pharmacological analysis of CFTR variants of cystic fibrosis using stem cell-derived organoids.
Cystic Fibrosis
Pharmacological approaches for targeting cystic fibrosis nonsense mutations.
Cystic Fibrosis
Pharmacological approaches to correcting the ion transport defect in cystic fibrosis.
Cystic Fibrosis
Pharmacological correction of a defect in PPAR-gamma signaling ameliorates disease severity in Cftr-deficient mice.
Cystic Fibrosis
Pharmacological Correction of Cystic Fibrosis: Molecular Mechanisms at the Plasma Membrane to Augment Mutant CFTR Function.
Cystic Fibrosis
Pharmacological Inhibition of the Ubiquitin Ligase RNF5 Rescues F508del-CFTR in Cystic Fibrosis Airway Epithelia.
Cystic Fibrosis
Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia.
Cystic Fibrosis
Pharmacological Rescue of Conditionally Reprogrammed Cystic Fibrosis Bronchial Epithelial Cells.
Cystic Fibrosis
Pharmacological Rescue of Ductal CFTR Rescue Pancreatic and Salivary Glands Acinar Cells and Tissue Function in Mouse Models of Autoimmune Diseases.
Cystic Fibrosis
Pharmacological reversal of renal cysts from secretion to absorption suggests a potential therapeutic strategy for managing autosomal dominant polycystic kidney disease.
Cystic Fibrosis
Pharmacological treatment of the biochemical defect in cystic fibrosis airways.
Cystic Fibrosis
Pharmacotherapy of the ion transport defect in cystic fibrosis: role of purinergic receptor agonists and other potential therapeutics.
Cystic Fibrosis
Phase I trial of intranasal and endobronchial administration of a recombinant adeno-associated virus serotype 2 (rAAV2)-CFTR vector in adult cystic fibrosis patients: a two-part clinical study.
Cystic Fibrosis
Phenotype and genotype of two Taiwanese cystic fibrosis siblings and a survey of delta F508 in East Asians.
Cystic Fibrosis
Phenotype-optimized sequence ensembles substantially improve prediction of disease-causing mutation in cystic fibrosis.
Cystic Fibrosis
Phenotypes of California CF Newborn Screen-Positive Children with CFTR 5T Allele by TG Repeat Length.
Cystic Fibrosis
Phenotypic and Genetic Characterization of Carbapenemase and ESBLs Producing Gram-negative Bacteria (GNB) Isolated from Patients with Cystic Fibrosis (CF) in Tehran Hospitals.
Cystic Fibrosis
Phenotypic characterization and comparison of Phe508del and cystic fibrosis transmembrane conductance regulator (CFTR) knockout rat models of cystic fibrosis generated by CRISPR/Cas9 gene editing.
Cystic Fibrosis
Phenotypic expression of genotype-phenotype correlation in cystic fibrosis patients carrying the 852del22 mutation.
Cystic Fibrosis
Phenotypic plasticity in gene expression and physiological response in red drum Sciaenops ocellatus exposed to a long-term freshwater environment.
Cystic Fibrosis
Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia.
Cystic Fibrosis
Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTATM.
Cystic Fibrosis
Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function.
Cystic Fibrosis
Phenylglycine and sulfonamide correctors of defective delta F508 and G551D cystic fibrosis transmembrane conductance regulator chloride-channel gating.
Cystic Fibrosis
Phenylhydrazones as Correctors of a Mutant Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
Phenylquinoxalinone CFTR activator as potential prosecretory therapy for constipation.
Cystic Fibrosis
Phloxine B interacts with the cystic fibrosis transmembrane conductance regulator at multiple sites to modulate channel activity.
Cystic Fibrosis
Phosphatase inhibitors activate normal and defective CFTR chloride channels.
Cystic Fibrosis
Phosphatidic acid metabolism regulates the intracellular trafficking and retrotranslocation of CFTR.
Cystic Fibrosis
Phosphatidylcholine Passes by Paracellular Transport to the Apical Side of the Polarized Biliary Tumor Cell Line Mz-ChA-1.
Cystic Fibrosis
Phosphodiesterase 4D forms a cAMP diffusion barrier at the apical membrane of the airway epithelium.
Cystic Fibrosis
Phosphorylated and ubiquitinated TDP-43 pathological inclusions in ALS and FTLD-U are recapitulated in SH-SY5Y cells.
Cystic Fibrosis
Phosphorylated C/EBP? influences a complex network involving YY1 and USF2 in lung epithelial cells.
Cystic Fibrosis
Phosphorylation by cAMP-dependent protein kinase causes a conformational change in the R domain of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Phosphorylation by protein kinase C is required for acute activation of cystic fibrosis transmembrane conductance regulator by protein kinase A.
Cystic Fibrosis
Phosphorylation of cystic fibrosis transmembrane conductance regulator (CFTR) serine-511 by the combined action of tyrosine kinases and CK2: the implication of tyrosine-512 and phenylalanine-508.
Cystic Fibrosis
Phosphorylation of P-glycoprotein by PKA and PKC modulates swelling-activated Cl- currents.
Cystic Fibrosis
Phosphorylation of PDZ1 domain attenuates NHERF-1 binding to cellular targets.
Cystic Fibrosis
Phosphorylation of protein kinase C sites in NBD1 and the R domain control CFTR channel activation by PKA.
Cystic Fibrosis
Phosphorylation of S955 at the protein kinase A consensus promotes maturation of the alpha subunit of the colonic H+,K+ -ATPase.
Cystic Fibrosis
Phosphorylation of the Chaperone-Like HspB5 Rescues Trafficking and Function of F508del-CFTR.
Cystic Fibrosis
Phosphorylation of vimentin is an intermediate step in protein kinase C-mediated glycoconjugate secretion.
Cystic Fibrosis
Phosphorylation-dependent 14-3-3 protein interactions regulate CFTR biogenesis.
Cystic Fibrosis
Phosphorylation-dependent block of cystic fibrosis transmembrane conductance regulator chloride channel by exogenous R domain protein.
Cystic Fibrosis
Phosphorylation-dependent modulation of CFTR macromolecular signalling complex activity by cigarette smoke condensate in airway epithelia.
Cystic Fibrosis
Phosphorylation-induced conformational changes of cystic fibrosis transmembrane conductance regulator monitored by attenuated total reflection-Fourier transform IR spectroscopy and fluorescence spectroscopy.
Cystic Fibrosis
Phosphorylation-regulated Cl- channel in CHO cells stably expressing the cystic fibrosis gene.
Cystic Fibrosis
Phylogenetic analysis of cystic fibrosis transmembrane conductance regulator gene in mammalian species argues for the development of a rabbit model for cystic fibrosis.
Cystic Fibrosis
Physiologic and molecular aspects of the Na+:HCO3- cotransporter in health and disease processes.
Cystic Fibrosis
Physiological and molecular mechanisms of osmoregulatory plasticity in killifish after seawater transfer.
Cystic Fibrosis
Physiological and Pathophysiological Relevance of the Anion Transporter Slc26a9 in Multiple Organs.
Cystic Fibrosis
Physiological evidence for a P2Y receptor responsive to diadenosine polyphosphates in human lung via Ca(2+) release studies in bronchial epithelial cells.
Cystic Fibrosis
Physiological impact of abnormal lipoxin A? production on cystic fibrosis airway epithelium and therapeutic potential.
Cystic Fibrosis
Physiological implications of the regulation of vacuolar H+-ATPase by chloride ions.
Cystic Fibrosis
Physiological modulation of CFTR activity by AMP-activated protein kinase in polarized T84 cells.
Cystic Fibrosis
Physiological regulation of ATP release at the apical surface of human airway epithelia.
Cystic Fibrosis
Physiological study of pulmonary involvement in adults with cystic fibrosis through simulated modeling of different clinical scenarios.
Cystic Fibrosis
Physiologically Based Pharmacokinetic Modeling of CFTR Modulation in People with Cystic Fibrosis Transitioning from Mono or Dual Regimens to Triple-Combination Elexacaftor/Tezacaftor/Ivacaftor.
Cystic Fibrosis
Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth.
Cystic Fibrosis
Pinacidil and levamisole prevent glutamate-induced death of hippocampal neuronal cells through reducing ROS production.
Cystic Fibrosis
Pitfall in the use of genotype analysis as the sole diagnostic criterion for cystic fibrosis.
Cystic Fibrosis
Pitfalls in the immunohistochemical localization of the cystic fibrosis transmembrane conductance regulator in paraffin embedded sweat glands.
Cystic Fibrosis
Pitfalls in the interpretation of CFTR variants in the context of incidental findings.
Cystic Fibrosis
PKA phosphorylation potentiates CFTR gating by relieving auto-inhibition on the stimulatory C terminus of the regulatory domain.
Cystic Fibrosis
PKC regulation of cardiac CFTR Cl- channel function in guinea pig ventricular myocytes.
Cystic Fibrosis
PKC-sensitive Cl- channels associated with ciliary epithelial homologue of pICln.
Cystic Fibrosis
Plasma Levels of the Bioactive Sphingolipid Metabolite S1P in Adult Cystic Fibrosis Patients: Potential Target for Immunonutrition?
Cystic Fibrosis
Plasma membrane Ca2+ ATPase isoform 2b interacts preferentially with Na+/H+ exchanger regulatory factor 2 in apical plasma membranes.
Cystic Fibrosis
Plasma membrane protein clusters appear in CFTR-expressing Xenopus laevis oocytes after cAMP stimulation.
Cystic Fibrosis
Plasma membrane-localized TMEM16 proteins are indispensable for expression of CFTR.
Cystic Fibrosis
Plasmodium falciparum-activated chloride channels are defective in erythrocytes from cystic fibrosis patients.
Cystic Fibrosis
Platelets: inflammatory effector cells in the conflagration of cystic fibrosis lung disease.
Cystic Fibrosis
Plumbagin Prevents Secretory Diarrhea by Inhibiting CaCC and CFTR Channel Activities.
Cystic Fibrosis
Pluripotent stem cells for disease modeling and drug screening: new perspectives for treatment of cystic fibrosis?
Cystic Fibrosis
Polar residues in membrane domains of proteins: molecular basis for helix-helix association in a mutant CFTR transmembrane segment.
Cystic Fibrosis
Polarization of specific tropomyosin isoforms in gastrointestinal epithelial cells and their impact on CFTR at the apical surface.
Cystic Fibrosis
Polarization-dependent apical membrane CFTR targeting underlies cAMP-stimulated Cl- secretion in epithelial cells.
Cystic Fibrosis
Polarized AAVR expression determines infectivity by AAV gene therapy vectors.
Cystic Fibrosis
Polarized distribution of HCO3- transport in human normal and cystic fibrosis nasal epithelia.
Cystic Fibrosis
Polarized distribution of key membrane transport proteins in the rat submandibular gland.
Cystic Fibrosis
Polarized expression of cAMP-activated chloride channels in isolated epithelial cells.
Cystic Fibrosis
Polarized expression of cystic fibrosis transmembrane conductance regulator and associated epithelial proteins during the regeneration of human airway surface epithelium in three-dimensional culture.
Cystic Fibrosis
Polarized signaling via purinoceptors in normal and cystic fibrosis airway epithelia.
Cystic Fibrosis
Poly thymidine polymorphism and cystic fibrosis in a non-Caucasian population.
Cystic Fibrosis
Polymorphic expression of multidrug resistance mRNA in lung parenchyma of nonpregnant and pregnant rats: a comparison to cystic fibrosis mRNA expression.
Cystic Fibrosis
Polymorphic markers suggest a gene flow of CFTR gene from Sub-Saharan/Arabian and Mediterranean to Brazilian Population.
Cystic Fibrosis
Polymorphism of cystic fibrosis gene in Japanese patients with chronic pancreatitis.
Cystic Fibrosis
Polymorphisms in the glutathione pathway modulate cystic fibrosis severity: a cross-sectional study.
Cystic Fibrosis
Polyvariant mutant cystic fibrosis transmembrane conductance regulator genes. The polymorphic (Tg)m locus explains the partial penetrance of the T5 polymorphism as a disease mutation.
Cystic Fibrosis
Population screening of F508del (DeltaF508), the most frequent mutation in the CFTR gene associated with cystic fibrosis in Argentina.
Cystic Fibrosis
Population-specific screening by mutation analysis for diseases frequent in Ashkenazi Jews.
Cystic Fibrosis
Porcine nasal epithelial cultures for studies of cystic fibrosis sinusitis.
Cystic Fibrosis
Pore dilation increases the bicarbonate permeability of CFTR, ANO1, and glycine receptor anion channels.
Cystic Fibrosis
Positional dependence of non-native polar mutations on folding of CFTR helical hairpins.
Cystic Fibrosis
Positional effects of premature termination codons on the biochemical and biophysical properties of CFTR.
Cystic Fibrosis
Positioning of extracellular loop 1 affects pore gating of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Positive charges at the intracellular mouth of the pore regulate anion conduction in the CFTR chloride channel.
Cystic Fibrosis
Positive epistasis between disease-causing missense mutations and silent polymorphism with effect on mRNA translation velocity.
Cystic Fibrosis
Positive neonatal screening for cystic fibrosis in neonates with renal failure.
Cystic Fibrosis
Post-Transcriptional Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Expression and Function by MicroRNAs.
Cystic Fibrosis
Post-translational disruption of the delta F508 cystic fibrosis transmembrane conductance regulator (CFTR)-molecular chaperone complex with geldanamycin stabilizes delta F508 CFTR in the rabbit reticulocyte lysate.
Cystic Fibrosis
Postnatal decrease of cystic fibrosis transmembrane conductance regulator gene expression in nasal epithelium of healthy newborn infants.
Cystic Fibrosis
Potent s-cis-locked bithiazole correctors of DeltaF508 cystic fibrosis transmembrane conductance regulator cellular processing for cystic fibrosis therapy.
Cystic Fibrosis
Potent, Metabolically Stable Benzopyrimido-pyrrolo-oxazine-dione (BPO) CFTR Inhibitors for Polycystic Kidney Disease.
Cystic Fibrosis
Potential genetic modifiers of the cystic fibrosis intestinal inflammatory phenotype on mouse chromosomes 1, 9, and 10.
Cystic Fibrosis
Potential of helper-dependent Adenoviral vectors in CRISPR-cas9-mediated lung gene therapy.
Cystic Fibrosis
Potential of Intestinal Current Measurement for Personalized Treatment of Patients with Cystic Fibrosis.
Cystic Fibrosis
Potential role for the common cystic fibrosis DeltaF508 mutation in Crohn's disease.
Cystic Fibrosis
Potential role of abnormal ion transport in the pathogenesis of chronic sinusitis.
Cystic Fibrosis
Potential role of CFTR in bisphenol A-induced malignant transformation of prostate cells via mitochondrial apoptosis.
Cystic Fibrosis
Potential role of the "NADPH oxidases" (NOX/DUOX) family in cystic fibrosis.
Cystic Fibrosis
Potentiation of cystic fibrosis transmembrane conductance regulator (CFTR) Cl- currents by the chemical solvent tetrahydrofuran.
Cystic Fibrosis
Potentiation of disease-associated CFTR mutants by hydrolyzable ATP analogs.
Cystic Fibrosis
Potentiation of effect of PKA stimulation of Xenopus CFTR by activation of PKC: role of NBD2.
Cystic Fibrosis
Potentiation of mutant CFTR Cl- channel currents by the naturally occurring stilbene compound resveratrol.
Cystic Fibrosis
Potentiation of the cystic fibrosis transmembrane conductance regulator by VX-770 involves stabilization of the pre-hydrolytic, O1 state.
Cystic Fibrosis
Potentiation of the cystic fibrosis transmembrane conductance regulator Cl- channel by ivacaftor is temperature-independent.
Cystic Fibrosis
Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis.
Cystic Fibrosis
Potentiators and Correctors in Paediatric Cystic Fibrosis Patients: A Narrative Review.
Cystic Fibrosis
Potentiators of Defective ?F508-CFTR Gating that Do Not Interfere with Corrector Action.
Cystic Fibrosis
PPAR{gamma} Agonists Inhibit Vasopressin Mediated Anion Transport in the MDCK-C7 Cell Line.
Cystic Fibrosis
Pre-clinical evaluation of three non-viral gene transfer agents for cystic fibrosis after aerosol delivery to the ovine lung.
Cystic Fibrosis
Precision toxicology shows that troxerutin alleviates ochratoxin A-induced renal lipotoxicity.
Cystic Fibrosis
Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis.
Cystic Fibrosis
Preclinical Studies of a Rare CF-Causing Mutation in the Second Nucleotide Binding Domain (c.3700A>G) Show Robust Functional Rescue in Primary Nasal Cultures by Novel CFTR Modulators.
Cystic Fibrosis
Predicting the risk of cystic fibrosis with echogenic fetal bowel and one cystic fibrosis mutation.
Cystic Fibrosis
Prediction of the antigenic sites of the cystic fibrosis transmembrane conductance regulator protein by molecular modelling.
Cystic Fibrosis
Predictive value of cystic fibrosis transmembrane conductance regulator (CFTR) in the diagnosis of gastric cancer.
Cystic Fibrosis
Pregnancy in cystic fibrosis: Review of the literature and expert recommendations.
Cystic Fibrosis
Preliminary evidence for cell membrane amelioration in children with cystic fibrosis by 5-MTHF and vitamin B12 supplementation: a single arm trial.
Cystic Fibrosis
Preliminary study of haplotypes linked to the rare cystic fibrosis E1104X mutation.
Cystic Fibrosis
Premature translation termination mutations are efficiently suppressed in a highly conserved region of yeast Ste6p, a member of the ATP-binding cassette (ABC) transporter family.
Cystic Fibrosis
Prenatal diagnosis of cystic fibrosis in different European populations: application of denaturing gradient gel electrophoresis.
Cystic Fibrosis
Preoperative serum methylation signature as prognostic tool after curative hepatectomy in patients with hepatocellular carcinoma.
Cystic Fibrosis
Prerenal azotemia from excessive sweating in an adult with a cystic fibrosis gene mutation.
Cystic Fibrosis
Presence of MRP8 and MRP14 in pancreatic cell lines: differential expression and localization in CFPAC-1 cells.
Cystic Fibrosis
Prevalence of ?F508 mutation in the cystic fibrosis transmembrane conductance regulator gene among cystic fibrosis patients from a Brazilian referral center.
Cystic Fibrosis
Prevalence of CBAVD in azoospermic men carrying pathogenic CFTR mutations - Evaluated in a cohort of 639 non-vasectomized azoospermic men.
Cystic Fibrosis
Prevalence of meconium ileus marks the severity of mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene.
Cystic Fibrosis
Prevention and treatment of secretory diarrhea by the lysophosphatidic acid analog Rx100.
Cystic Fibrosis
Prevention of chronic Pseudomonas aeruginosa infection in people with cystic fibrosis.
Cystic Fibrosis
Prevention of toxin-induced intestinal ion and fluid secretion by a small-molecule CFTR inhibitor.
Cystic Fibrosis
Previously unknown role for the ubiquitin ligase Ubr1 in endoplasmic reticulum-associated protein degradation.
Cystic Fibrosis
Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function.
Cystic Fibrosis
Pro-resolving lipid mediator Resolvin D1 serves as a marker of lung disease in cystic fibrosis.
Cystic Fibrosis
Pro-Secretory Activity and Pharmacology in Rabbits of an Aminophenyl-1,3,5-Triazine CFTR Activator for Dry Eye Disorders.
Cystic Fibrosis
Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant.
Cystic Fibrosis
Probiotics and Commensals Reverse TNF-alpha- and IFN-gamma-Induced Dysfunction in Human Intestinal Epithelial Cells.
Cystic Fibrosis
Processing Mutations Disrupt Interactions between the Nucleotide Binding and Transmembrane Domains of P-glycoprotein and the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Cystic Fibrosis
Processing mutations located throughout the human multidrug resistance P-glycoprotein disrupt interactions between the nucleotide binding domains.
Cystic Fibrosis
Processing of CFTR bearing the P574H mutation differs from wild-type and deltaF508-CFTR.
Cystic Fibrosis
Processing of CFTR: traversing the cellular maze--how much CFTR needs to go through to avoid cystic fibrosis?
Cystic Fibrosis
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive.
Cystic Fibrosis
Production and characterisation of monoclonal and polyclonal antibodies to different regions of the cystic fibrosis transmembrane conductance regulator (CFTR): detection of immunologically related proteins.
Cystic Fibrosis
Production and effects of alpha-melanocyte-stimulating hormone during acute lung injury.
Cystic Fibrosis
Production of cystic fibrosis transmembrane conductance regulator in the milk of transgenic mice.
Cystic Fibrosis
Profile of tezacaftor/ivacaftor combination and its potential in the treatment of cystic fibrosis.
Cystic Fibrosis
Progress toward generating a ferret model of cystic fibrosis by somatic cell nuclear transfer.
Cystic Fibrosis
Progression of early structural lung disease in young children with cystic fibrosis assessed using CT.
Cystic Fibrosis
Proinflammatory Cytokine Secretion is Suppressed by TMEM16A or CFTR Channel Activity in Human Cystic Fibrosis Bronchial Epithelia.
Cystic Fibrosis
Proliferation, not apoptosis, alters epithelial cell migration in small intestine of CFTR null mice.
Cystic Fibrosis
Prolonged administration of secretin to normal rats increases biliary proliferation and secretin-induced ductal secretory activity.
Cystic Fibrosis
Prolonged treatment of cells with genistein modulates the expression and function of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Promoter hypermethylation of the CFTR gene and clinical/pathological features associated with non-small cell lung cancer.
Cystic Fibrosis
Promoter hypermethylation of the CFTR gene as a novel diagnostic and prognostic marker of breast cancer.
Cystic Fibrosis
Properties of a Cl(-)-conductive pathway(s) in microsomes from rat kidney inner medulla. Involvement of cystic fibrosis transmembrane regulator protein.
Cystic Fibrosis
Properties of cAMP-dependent and Ca(2+)-dependent whole cell Cl- conductances in rat epididymal cells.
Cystic Fibrosis
Properties of CFTR activated by the xanthine derivative X-33 in human airway Calu-3 cells.
Cystic Fibrosis
Properties of chloride-conductive pathways in rat kidney cortical and outer-medulla brush-border membranes--inhibition by anti-(cystic fibrosis transmembrane regulator) mAbs.
Cystic Fibrosis
Properties of the cAMP-activated C1- current in choroid plexus epithelial cells isolated from the rat.
Cystic Fibrosis
Propofol inhibits carbachol-induced chloride secretion by directly targeting the basolateral K(+) channel in rat ileum epithelium.
Cystic Fibrosis
Proportion of cystic fibrosis gene mutations not detected by routine testing in men with obstructive azoospermia.
Cystic Fibrosis
Prospective analysis of cystic fibrosis transmembrane regulator mutations in adults with bronchiectasis or pulmonary nontuberculous mycobacterial infection.
Cystic Fibrosis
Prospective and parallel assessments of cystic fibrosis newborn screening protocols in the Czech Republic: IRT/DNA/IRT versus IRT/PAP and IRT/PAP/DNA.
Cystic Fibrosis
Prostacyclin receptor-mediated ATP release from erythrocytes requires the voltage-dependent anion channel.
Cystic Fibrosis
Prostaglandin E2 induces chloride secretion through crosstalk between cAMP and calcium signaling in mouse inner medullary collecting duct cells.
Cystic Fibrosis
Prostaglandin E2 stimulates anion and fluid secretion triggered by lipopolysaccharide in rat vaginal epithelium.
Cystic Fibrosis
Prostaglandin F2alpha stimulates CFTR activity by PKA- and PKC-dependent phosphorylation.
Cystic Fibrosis
Prostanoids secreted by alveolar macrophages enhance ionic currents in swine tracheal submucosal gland cells.
Cystic Fibrosis
Protease-activated receptor 2 mediates mucus secretion in the airway submucosal gland.
Cystic Fibrosis
Protease-activated receptor-1 stimulates Ca(2+)-dependent Cl(-) secretion in human intestinal epithelial cells.
Cystic Fibrosis
Proteases and antiproteases in cystic fibrosis: pathogenetic considerations and therapeutic strategies.
Cystic Fibrosis
Proteasome-dependent pharmacological rescue of cystic fibrosis transmembrane conductance regulator revealed by mutation of glycine 622.
Cystic Fibrosis
Protection against bronchial asthma by CFTR delta F508 mutation: a heterozygote advantage in cystic fibrosis.
Cystic Fibrosis
Protection against oxidative stress in beta thalassemia/hemoglobin E erythrocytes by inhibitors of glutathione efflux transporters.
Cystic Fibrosis
Protection of Cftr knockout mice from acute lung infection by a helper-dependent adenoviral vector expressing Cftr in airway epithelia.
Cystic Fibrosis
Protective role of cardiac CFTR activation upon early reperfusion against myocardial infarction.
Cystic Fibrosis
Protein folding. Translational tuning optimizes nascent protein folding in cells.
Cystic Fibrosis
Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites.
Cystic Fibrosis
Protein kinase A associates with cystic fibrosis transmembrane conductance regulator via an interaction with ezrin.
Cystic Fibrosis
Protein kinase C activates chloride conductance in C127 cells stably expressing the cystic fibrosis gene.
Cystic Fibrosis
Protein kinase C and Ca2+ activation of mucin secretion in airway goblet cells.
Cystic Fibrosis
Protein kinase C epsilon-dependent regulation of cystic fibrosis transmembrane regulator involves binding to a receptor for activated C kinase (RACK1) and RACK1 binding to Na+/H+ exchange regulatory factor.
Cystic Fibrosis
Protein kinase C mediates down-regulation of cystic fibrosis transmembrane conductance regulator levels in epithelial cells.
Cystic Fibrosis
Protein kinase C phosphorylation disrupts Na+/H+ exchanger regulatory factor 1 autoinhibition and promotes cystic fibrosis transmembrane conductance regulator macromolecular assembly.
Cystic Fibrosis
Protein kinase C regulates the magnitude and stability of CFTR currents in pancreatic duct cells.
Cystic Fibrosis
Protein kinase CK2, cystic fibrosis transmembrane conductance regulator, and the deltaF508 mutation: F508 deletion disrupts a kinase-binding site.
Cystic Fibrosis
Protein kinase-independent activation of CFTR by phosphatidylinositol phosphates.
Cystic Fibrosis
Protein phosphatase 1 coordinates CFTR-dependent airway epithelial HCO3 (-) secretion by reciprocal regulation of apical and basolateral membrane Cl(-) -HCO3 (-) exchangers.
Cystic Fibrosis
Protein phosphatase 2C dephosphorylates and inactivates cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Protein trafficking rates assessed by quantum dot quenching with bromocresol green.
Cystic Fibrosis
Proteinuria in cystic fibrosis: a possible correlation between genotype and renal phenotype.
Cystic Fibrosis
Proteome of the porosome complex in human airway epithelia: interaction with the cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
Proteomic and ionomic profiling reveals significant alterations of protein expression and calcium homeostasis in cystic fibrosis cells.
Cystic Fibrosis
Proteomics investigation of human platelets in healthy donors and cystic fibrosis patients by shotgun nUPLC-MSE and 2DE: a comparative study.
Cystic Fibrosis
Protocol for Application, Standardization and Validation of the Forskolin-Induced Swelling Assay in Cystic Fibrosis Human Colon Organoids.
Cystic Fibrosis
Proton leak and CFTR in regulation of Golgi pH in respiratory epithelial cells.
Cystic Fibrosis
PRSS1 intron mutations in patients with pancreatic cancer and chronic pancreatitis.
Cystic Fibrosis
Pseudomonas aeruginosa alginate promotes Burkholderia cenocepacia persistence in cystic fibrosis transmembrane conductance regulator knockout mice.
Cystic Fibrosis
Pseudomonas aeruginosa Cif Defines a Distinct Class of ?/? Epoxide Hydrolases Utilizing a His/Tyr Ring-Opening Pair.
Cystic Fibrosis
Pseudomonas aeruginosa Cif protein enhances the ubiquitination and proteasomal degradation of the transporter associated with antigen processing (TAP) and reduces major histocompatibility complex (MHC) class I antigen presentation.
Cystic Fibrosis
Pseudomonas aeruginosa exploits lipid A and muropeptides modification as a strategy to lower innate immunity during cystic fibrosis lung infection.
Cystic Fibrosis
Pseudomonas aeruginosa Homoserine Lactone Activates Store-operated cAMP and Cystic Fibrosis Transmembrane Regulator-dependent Cl- Secretion by Human Airway Epithelia.
Cystic Fibrosis
Pseudomonas aeruginosa induction of apoptosis in respiratory epithelial cells: analysis of the effects of cystic fibrosis transmembrane conductance regulator dysfunction and bacterial virulence factors.
Cystic Fibrosis
Pseudomonas aeruginosa internalization by human epithelial respiratory cells depends on cell differentiation, polarity, and junctional complex integrity.
Cystic Fibrosis
Pseudomonas aeruginosa LasB protease impairs innate immunity in mice and humans by targeting a lung epithelial cystic fibrosis transmembrane regulator-IL-6-antimicrobial-repair pathway.
Cystic Fibrosis
Pseudomonas aeruginosa lipopolysaccharide induces CF-like alteration of protein secretion by human tracheal gland cells.
Cystic Fibrosis
Pseudomonas aeruginosa pyocyanin inactivates lung epithelial vacuolar ATPase-dependent cystic fibrosis transmembrane conductance regulator expression and localization.
Cystic Fibrosis
Pseudomonas aeruginosa sabotages the generation of host proresolving lipid mediators.
Cystic Fibrosis
Pseudomonas aeruginosa triggers CFTR-mediated airway surface liquid secretion in swine trachea.
Cystic Fibrosis
Pseudomonas aeruginosa-induced apoptosis is defective in respiratory epithelial cells expressing mutant cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Pseudomonas Aeruginosa: Genetic Adaptation, A Strategy for its Persistence in Cystic Fibrosis.
Cystic Fibrosis
pSURF-2, a modified BAC vector for selective YAC cloning and functional analysis.
Cystic Fibrosis
Pulmonary bacterial pathogens in cystic fibrosis patients and antibiotic therapy: a tool for the health workers.
Cystic Fibrosis
Pulmonary Mycobacterium avium complex infection associated with the IVS8-T5 allele of the CFTR gene.
Cystic Fibrosis
Pulmonary nontuberculous mycobacterial disease: prospective study of a distinct preexisting syndrome.
Cystic Fibrosis
Purification and characterization of recombinant cystic fibrosis transmembrane conductance regulator from Chinese hamster ovary and insect cells.
Cystic Fibrosis
Purification and crystallization of the cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
Purification and reconstitution of epithelial chloride channel cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Purification of CFTR for mass spectrometry analysis: identification of palmitoylation and other post-translational modifications.
Cystic Fibrosis
Purification of the cystic fibrosis transmembrane conductance regulator protein expressed in Saccharomyces cerevisiae.
Cystic Fibrosis
Purification, crystallization and preliminary X-ray diffraction analysis of Cif, a virulence factor secreted by Pseudomonas aeruginosa.
Cystic Fibrosis
Purified cystic fibrosis transmembrane conductance regulator (CFTR) does not function as an ATP channel.
Cystic Fibrosis
Purine release from spinal cord microglia after elevation of calcium by glutamate.
Cystic Fibrosis
Purinergic signaling underlies CFTR control of human airway epithelial cell volume.
Cystic Fibrosis
Purinoceptor-coupled Cl- channels in mouse heart: a novel, alternative pathway for CFTR regulation.
Cystic Fibrosis
Putative interaction of brush cells with bicarbonate secreting cells in the proximal corpus mucosa.
Cystic Fibrosis
Pyridine nucleotide redox potential modulates cystic fibrosis transmembrane conductance regulator Cl- conductance.
Cystic Fibrosis
Pyrophosphate stimulates wild-type and mutant cystic fibrosis transmembrane conductance regulator Cl- channels.
Cystic Fibrosis
Qualitative and quantitative analysis of mRNA associated with four putative splicing mutations (621+3A-->G, 2751+2T-->A, 296+1G-->C, 1717-9T-->C-D565G) and one nonsense mutation (E822X) in the CFTR gene.
Cystic Fibrosis
Quantification of CFTR splice variants in adults with disseminated bronchiectasis, using the TaqMan fluorogenic detection system.
Cystic Fibrosis
Quantification of Phenotypic Variability of Lung Disease in Children with Cystic Fibrosis.
Cystic Fibrosis
Quantitative analysis of the packaging capacity of recombinant adeno-associated virus.
Cystic Fibrosis
Quantitative Evaluation of CFTR Pre-mRNA Splicing Dependent on the (TG)mTn Poly-Variant Tract.
Cystic Fibrosis
Quantitative Method for the Analysis of Ivacaftor, Hydroxymethyl Ivacaftor, Ivacaftor Carboxylate, Lumacaftor, and Tezacaftor in Plasma and Sputum Using Liquid Chromatography With Tandem Mass Spectrometry and Its Clinical Applicability.
Cystic Fibrosis
Quantitative phase imaging to study transmembrane water fluxes regulated by CFTR and AQP3 in living human airway epithelial CFBE cells and CHO cells.
Cystic Fibrosis
QUANTUM DOT SINGLE MOLECULE TRACKING REVEALS A WIDE RANGE OF DIFFUSIVE MOTIONS OF MEMBRANE TRANSPORT PROTEINS.
Cystic Fibrosis
Quercetin increases cystic fibrosis transmembrane conductance regulator-mediated chloride transport and ciliary beat frequency: therapeutic implications for chronic rhinosinusitis.
Cystic Fibrosis
R-domain interactions with distal regions of CFTR lead to phosphorylation and activation.
Cystic Fibrosis
R-Domain Phosphorylation by Protein Kinase A Stimulates Dissociation of Unhydrolyzed ATP from the First Nucleotide-Binding Site of the Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
R248G cystic fibrosis transmembrane conductance regulator mutation in three siblings presenting with recurrent acute pancreatitis and reproductive issues: a case series.
Cystic Fibrosis
Rab11b regulates the apical recycling of the cystic fibrosis transmembrane conductance regulator in polarized intestinal epithelial cells.
Cystic Fibrosis
Rab27a negatively regulates CFTR chloride channel function in colonic epithelia: involvement of the effector proteins in the regulatory mechanism.
Cystic Fibrosis
Rabbit erythrocytes possess adenylyl cyclase type II that is activated by the heterotrimeric G proteins Gs and Gi.
Cystic Fibrosis
Rabbit pancreatic acini express CFTR as a cAMP-activated chloride efflux pathway.
Cystic Fibrosis
Racially equitable diagnosis of cystic fibrosis using next-generation DNA sequencing: a case report.
Cystic Fibrosis
RACK1 interacts with filamin-A to regulate plasma membrane levels of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Ractopamine, a livestock feed additive, is a full agonist at trace amine-associated receptor 1.
Cystic Fibrosis
Radioisotopic imaging allows optimization of adenovirus lung deposition for cystic fibrosis gene therapy.
Cystic Fibrosis
Radiological analysis of children with cystic fibrosis who are homozygous for cystic fibrosis transmembrane conductance regulator mutation S549R (T-->G).
Cystic Fibrosis
Radiotracer studies of cystic fibrosis transmembrane conductance regulator expressed in Xenopus oocytes.
Cystic Fibrosis
Rapid and reliable genotyping of polymorphic loci modifying correct splicing of CFTR pre-mRNA using mass spectrometry.
Cystic Fibrosis
Rapid detection of cystic fibrosis transmembrane conductance regulator gene IVS8 5T variant by real-time PCR.
Cystic Fibrosis
Rapid endocytosis of the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Rapid quantitation of gene therapy specific CFTR expression using the amplification refractory mutation system.
Cystic Fibrosis
Rapid screening for 31 mutations and polymorphisms in the cystic fibrosis transmembrane conductance regulator gene by Lminex xMAP suspension array.
Cystic Fibrosis
Rapid screening of genetic polymorphisms using buccal cell DNA with detection by matrix-assisted laser desorption/ionization mass spectrometry.
Cystic Fibrosis
Rare association between cystic fibrosis, Chiari I malformation, and hydrocephalus in a baby: a case report and review of the literature.
Cystic Fibrosis
Rat brain p64H1, expression of a new member of the p64 chloride channel protein family in endoplasmic reticulum.
Cystic Fibrosis
Rational approach to genetic testing of cystic fibrosis (CF) in infertile men.
Cystic Fibrosis
Rational Coupled Dynamics Network Manipulation Rescues Disease-Relevant Mutant Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
Rational Design of Potent and Selective Inhibitors of an Epoxide Hydrolase Virulence Factor from Pseudomonas aeruginosa.
Cystic Fibrosis
Rattlesnake Phospholipase A2 Increases CFTR-Chloride Channel Current and Corrects ?F508CFTR Dysfunction: Impact in Cystic Fibrosis.
Cystic Fibrosis
Re: Loss of Cystic Fibrosis Transmembrane Regulator Impairs Intestinal Oxalate Secretion.
Cystic Fibrosis
Re: Lu et al.: Different cystic fibrosis transmembrane conductance regulator mutations in Chinese men with congenital bilateral absence of the vas deferens and other acquired obstructive azoospermia (Urology 2013;82:824-828).
Cystic Fibrosis
Reactive oxygen nitrogen species decrease cystic fibrosis transmembrane conductance regulator expression and cAMP-mediated Cl- secretion in airway epithelia.
Cystic Fibrosis
Readministration of helper-dependent adenoviral vectors to mouse airway mediated via transient immunosuppression.
Cystic Fibrosis
Real-World Long-Term Ivacaftor for Cystic Fibrosis in France: Clinical Effectiveness and Healthcare Resource Utilization.
Cystic Fibrosis
Real-World Safety of CFTR Modulators in the Treatment of Cystic Fibrosis: A Systematic Review.
Cystic Fibrosis
Reappraisal of Frequency of Common Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations in Iranian Cystic Fibrosis Patients.
Cystic Fibrosis
Recent advances and new perspectives in targeting CFTR for therapy of cystic fibrosis and enterotoxin-induced secretory diarrheas.
Cystic Fibrosis
Recent advances in the early treatment of cystic fibrosis: Bridging the gap to highly effective modulator therapy.
Cystic Fibrosis
Recent advances in understanding and managing cystic fibrosis transmembrane conductance regulator dysfunction.
Cystic Fibrosis
Recent Progress in CFTR Interactome Mapping and Its Importance for Cystic Fibrosis.
Cystic Fibrosis
Recent Progress in the Discovery and Development of Small-Molecule Modulators of CFTR.
Cystic Fibrosis
Receptor activity modifying proteins regulate the activity of a calcitonin gene-related peptide receptor in rabbit aortic endothelial cells.
Cystic Fibrosis
Receptor-mediated activation of the heterotrimeric G-protein Gs results in ATP release from erythrocytes.
Cystic Fibrosis
Receptors that couple to 2 classes of G proteins increase cAMP and activate CFTR expressed in Xenopus oocytes.
Cystic Fibrosis
Reciprocal protein kinase A regulatory interactions between cystic fibrosis transmembrane conductance regulator and Na+/H+ exchanger isoform 3 in a renal polarized epithelial cell model.
Cystic Fibrosis
Recognition of misfolding proteins by PA700, the regulatory subcomplex of the 26 S proteasome.
Cystic Fibrosis
Recombinant adenovirus deleted of all viral genes for gene therapy of cystic fibrosis.
Cystic Fibrosis
Recombinant synthesis of cystic fibrosis transmembrane conductance regulator and functional nucleotide-binding domains.
Cystic Fibrosis
Recombinant synthesis, purification, and nucleotide binding characteristics of the first nucleotide binding domain of the cystic fibrosis gene product.
Cystic Fibrosis
Recommendations for the classification of diseases as CFTR-related disorders.
Cystic Fibrosis
Reconstitution of a chemical defense signaling pathway in a heterologous system.
Cystic Fibrosis
Recovery of Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction after Smoking Cessation.
Cystic Fibrosis
Recovery of airway cystic fibrosis transmembrane conductance regulator function in mice with cystic fibrosis after single-dose lentivirus-mediated gene transfer.
Cystic Fibrosis
Rectal epithelial expression of protein kinase A phosphorylation of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Rectification of cystic fibrosis transmembrane conductance regulator chloride channel mediated by extracellular divalent cations.
Cystic Fibrosis
Rectification of whole cell cystic fibrosis transmembrane conductance regulator chloride current.
Cystic Fibrosis
Red Blood Cells Under Flow Show Maximal ATP Release for Specific Hematocrit.
Cystic Fibrosis
Redistribution of immunofluorescence of CFTR anion channel and NKCC cotransporter in chloride cells during adaptation of the killifish Fundulus heteroclitus to sea water.
Cystic Fibrosis
Redox reagents and divalent cations alter the kinetics of cystic fibrosis transmembrane conductance regulator channel gating.
Cystic Fibrosis
Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung.
Cystic Fibrosis
Reduced Arylsulfatase B activity in leukocytes from cystic fibrosis patients.
Cystic Fibrosis
Reduced blood pressure of CFTR-F508del carriers correlates with diminished arterial reactivity rather than circulating blood volume in mice.
Cystic Fibrosis
Reduced caveolin-1 promotes hyperinflammation due to abnormal heme oxygenase-1 localization in lipopolysaccharide-challenged macrophages with dysfunctional cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Reduced expression of the cystic fibrosis transmembrane conductance regulator gene in the hypothalamus of patients with Alzheimer's disease.
Cystic Fibrosis
Reduced GM1 Ganglioside in CFTR-Deficient Human Airway Cells Results in Decreased ss1-integrin Signaling and Delayed Wound Repair.
Cystic Fibrosis
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis.
Cystic Fibrosis
Reduced IL-10 secretion by CD4+ T lymphocytes expressing mutant cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
Reduced Intestinal Inflammation with Lumacaftor/Ivacaftor in Adolescents with Cystic Fibrosis.
Cystic Fibrosis
Reduced neutrophil elastase inhibitor elafin and elevated transforming growth factor-?1 are linked to inflammatory response in sputum of cystic fibrosis patients with Pseudomonas aeruginosa.
Cystic Fibrosis
Reduced NHE3-mediated Na+ absorption increases survival and decreases the incidence of intestinal obstructions in cystic fibrosis mice.
Cystic Fibrosis
Reduced PDZ interactions of rescued ?F508CFTR increases its cell surface mobility.
Cystic Fibrosis
Redundancy of mammalian proteasome beta subunit function during endoplasmic reticulum associated degradation.
Cystic Fibrosis
Refining the continuum of CFTR-associated disorders in the era of newborn screening.
Cystic Fibrosis
Regulatable promoters for use in gene therapy applications: modification of the 5'-flanking region of the CFTR gene with multiple cAMP response elements to support basal, low-level gene expression that can be upregulated by exogenous agents that raise intracellular levels of cAMP.
Cystic Fibrosis
Regulated recycling of mutant CFTR is partially restored by pharmacological treatment.
Cystic Fibrosis
Regulated traffic of anion transporters in mammalian Brunner's glands: a role for water and fluid transport.
Cystic Fibrosis
Regulation and properties of KCNQ1 (K(V)LQT1) and impact of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Regulation by ATP and ADP of CFTR chloride channels that contain mutant nucleotide-binding domains.
Cystic Fibrosis
Regulation of a human chloride channel. a paradigm for integrating input from calcium, type ii calmodulin-dependent protein kinase, and inositol 3,4,5,6-tetrakisphosphate.
Cystic Fibrosis
Regulation of activation and processing of the cystic fibrosis transmembrane conductance regulator (CFTR) by a complex electrostatic interaction between the regulatory domain and cytoplasmic loop 3.
Cystic Fibrosis
Regulation of adenovirus-mediated transgene expression by the viral E4 gene products: requirement for E4 ORF3.
Cystic Fibrosis
Regulation of amiloride-sensitive sodium absorption in murine airway epithelium by C-type natriuretic peptide.
Cystic Fibrosis
Regulation of antiprotease and antimicrobial protein secretion by airway submucosal gland serous cells.
Cystic Fibrosis
Regulation of apical surface fluid and protein secretion in human airway epithelial cell line Calu-3.
Cystic Fibrosis
Regulation of Ca(2+)-activated chloride channels by cAMP and CFTR in parotid acinar cells.
Cystic Fibrosis
Regulation of cAMP-dependent chloride channels in DC1 immortalized rabbit distal tubule cells in culture.
Cystic Fibrosis
Regulation of CFTR Bicarbonate Channel Activity by WNK1: Implications for Pancreatitis and CFTR-Related Disorders.
Cystic Fibrosis
Regulation of CFTR Biogenesis by the Proteostatic Network and Pharmacological Modulators.
Cystic Fibrosis
Regulation of CFTR Chloride Channel Macroscopic Conductance by Extracellular Bicarbonate.
Cystic Fibrosis
Regulation of channel gating by AMP-activated protein kinase modulates cystic fibrosis transmembrane conductance regulator activity in lung submucosal cells.
Cystic Fibrosis
Regulation of chemokine expression by NaCl occurs independently of cystic fibrosis transmembrane conductance regulator in macrophages.
Cystic Fibrosis
Regulation of chloride secretion across porcine endometrial epithelial cells by prostaglandin E2.
Cystic Fibrosis
Regulation of Cl- Electrolyte Permeability in Epithelia by Active Traditional Chinese Medicine Monomers for Diarrhea.
Cystic Fibrosis
Regulation of Cl- secretion by alpha2-adrenergic receptors in mouse colonic epithelium.
Cystic Fibrosis
Regulation of Cl-/ HCO3- exchange by cystic fibrosis transmembrane conductance regulator expressed in NIH 3T3 and HEK 293 cells.
Cystic Fibrosis
Regulation of conductance by the number of fixed positive charges in the intracellular vestibule of the CFTR chloride channel pore.
Cystic Fibrosis
Regulation of cortical structure by the ezrin-radixin-moesin protein family.
Cystic Fibrosis
Regulation of cystic fibrosis transmembrane conductance regulator (CFTR) gene transcription and alternative RNA splicing in a model of developing intestinal epithelium.
Cystic Fibrosis
Regulation of Cystic Fibrosis Transmembrane Conductance Regulator by MicroRNA-145, -223, and -494 Is Altered in ?F508 Cystic Fibrosis Airway Epithelium.
Cystic Fibrosis
Regulation of cystic fibrosis transmembrane conductance regulator single-channel gating by bivalent PDZ-domain-mediated interaction.
Cystic Fibrosis
Regulation of cystic fibrosis transmembrane regulator trafficking and protein expression by a Rho family small GTPase TC10.
Cystic Fibrosis
Regulation of electrogenic anion secretion in normal and cystic fibrosis gallbladder mucosa.
Cystic Fibrosis
Regulation of endocytic trafficking and acidification are independent of the cystic fibrosis transmembrane regulator.
Cystic Fibrosis
REGULATION OF ENDOGENOUS ENaC FUNCTIONAL EXPRESSION BY CFTR AND {Delta}F508-CFTR IN AIRWAY EPITHELIAL CELLS.
Cystic Fibrosis
Regulation of epithelial ion channels by the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Regulation of extracellular UTP-activated Cl- current by P2Y-PLC-PKC signaling and ATP hydrolysis in mouse ventricular myocytes.
Cystic Fibrosis
Regulation of gap junctional communication by a pro-inflammatory cytokine in cystic fibrosis transmembrane conductance regulator-expressing but not cystic fibrosis airway cells.
Cystic Fibrosis
Regulation of hepatic ABCC transporters by xenobiotics and in disease states.
Cystic Fibrosis
Regulation of hepatic sulfotransferase (SULT) 1E1 expression and effects on estrogenic activity in cystic fibrosis (CF).
Cystic Fibrosis
Regulation of human cystic fibrosis transmembrane conductance regulator (CFTR) by serum- and glucocorticoid-inducible kinase (SGK1).
Cystic Fibrosis
Regulation of intestinal gene expression by dietary zinc: induction of uroguanylin mRNA by zinc deficiency.
Cystic Fibrosis
Regulation of murine cystic fibrosis transmembrane conductance regulator Cl- channels expressed in Chinese hamster ovary cells.
Cystic Fibrosis
Regulation of Pseudomonas aeruginosa internalization after contact lens wear in vivo and in serum-free culture by ocular surface cells.
Cystic Fibrosis
Regulation of recombinant cardiac cystic fibrosis transmembrane conductance regulator chloride channels by protein kinase C.
Cystic Fibrosis
Regulation of the Coxsackie and adenovirus receptor expression is dependent on cystic fibrosis transmembrane regulator in airway epithelial cells.
Cystic Fibrosis
Regulation of the cystic fibrosis transmembrane conductance regulator anion channel by tyrosine phosphorylation.
Cystic Fibrosis
Regulation of the cystic fibrosis transmembrane conductance regulator channel by beta-adrenergic agonists and vasoactive intestinal peptide in rat smooth muscle cells and its role in vasorelaxation.
Cystic Fibrosis
Regulation of the cystic fibrosis transmembrane conductance regulator chloride channel by MgATP.
Cystic Fibrosis
Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by its R domain.
Cystic Fibrosis
Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by negative charge in the R domain.
Cystic Fibrosis
Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by specific protein kinases and protein phosphatases.
Cystic Fibrosis
Regulation of the gating of cystic fibrosis transmembrane conductance regulator C1 channels by phosphorylation and ATP hydrolysis.
Cystic Fibrosis
Regulation of the ROMK channel: interaction of the ROMK with associate proteins.
Cystic Fibrosis
Regulation of Ubiquitin-Proteasome System mediated Degradation by Cytosolic Stress.
Cystic Fibrosis
Regulation of vascular tone and arterial blood pressure: role of chloride transport in vascular smooth muscle.
Cystic Fibrosis
Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators.
Cystic Fibrosis
Regulatory dynamics of 11p13 suggest a role for EHF in modifying CF lung disease severity.
Cystic Fibrosis
Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTR.
Cystic Fibrosis
Regulatory interaction between the cystic fibrosis transmembrane conductance regulator and HCO3- salvage mechanisms in model systems and the mouse pancreatic duct.
Cystic Fibrosis
Regulatory interactions of N1303K-CFTR and ENaC in Xenopus oocytes: evidence that chloride transport is not necessary for inhibition of ENaC.
Cystic Fibrosis
Regulatory mechanism underlying cyclic changes in mouse uterine bicarbonate secretion: role of estrogen.
Cystic Fibrosis
Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions.
Cystic Fibrosis
Relating the Disease Mutation Spectrum to the Evolution of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Cystic Fibrosis
Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis.
Cystic Fibrosis
Relation between the anatomical genital phenotype and cystic fibrosis transmembrane conductance regulator gene mutations in the absence of the vas deferens.
Cystic Fibrosis
Relations between the frequency of the DeltaF 508 mutation and the course of pulmonary disease in cystic fibrosis patients infected with Pseudomonas aeruginosa.
Cystic Fibrosis
Relationship between anion binding and anion permeability revealed by mutagenesis within the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Cystic Fibrosis
Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Relationship of a non-cystic fibrosis transmembrane conductance regulator-mediated chloride conductance to organ-level disease in Cftr(-/-) mice.
Cystic Fibrosis
Relationships among CFTR expression, HCO3- secretion, and host defense may inform gene- and cell-based cystic fibrosis therapies.
Cystic Fibrosis
Relationships between cystic fibrosis transmembrane conductance regulator, extracellular nucleotides and cystic fibrosis.
Cystic Fibrosis
Relative contribution of clear cells and principal cells to luminal pH in the mouse epididymis.
Cystic Fibrosis
Relative contribution of different transmembrane segments to the CFTR chloride channel pore.
Cystic Fibrosis
Relative Movements of Transmembrane Regions at the Outer Mouth of the Cystic Fibrosis Transmembrane Conductance Regulator Channel Pore during Channel Gating.
Cystic Fibrosis
Release of ATP from retinal pigment epithelial cells involves both CFTR and vesicular transport.
Cystic Fibrosis
Relevance of genetic counselling in couples prior to intracytoplasmic sperm injection.
Cystic Fibrosis
Relevance of variants in serum antiproteinases for the course of chronic pancreatitis.
Cystic Fibrosis
Reliability of polymerase chain reaction (PCR) analysis of single cells for preimplantation genetic diagnosis.
Cystic Fibrosis
Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis.
Cystic Fibrosis
Removal of the Fe(iii) site promotes activation of the human cystic fibrosis transmembrane conductance regulator by high-affinity Zn(ii) binding.
Cystic Fibrosis
Renal Medullary Interstitial COX-2 (Cyclooxygenase-2) Is Essential in Preventing Salt-Sensitive Hypertension and Maintaining Renal Inner Medulla/Papilla Structural Integrity.
Cystic Fibrosis
Repairing mutated proteins--development of small molecules targeting defects in the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Repairing the basic defect in cystic fibrosis - one approach is not enough.
Cystic Fibrosis
Repeat administration of an adenovirus vector encoding cystic fibrosis transmembrane conductance regulator to the nasal epithelium of patients with cystic fibrosis.
Cystic Fibrosis
Repeat administration of DNA/liposomes to the nasal epithelium of patients with cystic fibrosis.
Cystic Fibrosis
Repeatability and Diagnostic Value of Nasal Potential Difference in a Genetically Admixed Population.
Cystic Fibrosis
Repeated adeno-associated virus serotype 2 aerosol-mediated cystic fibrosis transmembrane regulator gene transfer to the lungs of patients with cystic fibrosis: a multicenter, double-blind, placebo-controlled trial.
Cystic Fibrosis
Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial.
Cystic Fibrosis
Report of a Korean patient with cystic fibrosis, carrying Q98R and Q220X mutations in the CFTR gene.
Cystic Fibrosis
Report of two patients with associated conditions in addition to cystic fibrosis.
Cystic Fibrosis
Report on the p.Ser489X (p.Ser489*) CFTR mutation, a variant with severe associated phenotype and high prevalence in a Quebec French-Canadian cystic fibrosis patient population.
Cystic Fibrosis
Rescue from Pseudomonas aeruginosa Airway Infection via Stem Cell Transplantation.
Cystic Fibrosis
Rescue of CFTR NBD2 mutants N1303K and S1235R is influenced by the functioning of the autophagosome.
Cystic Fibrosis
Rescue of defective ATP8B1 trafficking by CFTR correctors as a therapeutic strategy for familial intrahepatic cholestasis.
Cystic Fibrosis
Rescue of DeltaF508 and other misprocessed CFTR mutants by a novel quinazoline compound.
Cystic Fibrosis
Rescue of DeltaF508-CFTR (cystic fibrosis transmembrane conductance regulator) by curcumin: involvement of the keratin 18 network.
Cystic Fibrosis
Rescue of F508del-CFTR by RXR motif inactivation triggers proteome modulation associated with the unfolded protein response.
Cystic Fibrosis
Rescue of Functional CFTR Channels in Cystic Fibrosis: A Dramatic Multivalent Effect Using Iminosugar Cluster-Based Correctors.
Cystic Fibrosis
Rescue of functional DeltaF508-CFTR channels by co-expression with truncated CFTR constructs in COS-1 cells.
Cystic Fibrosis
Rescue of functional F508del cystic fibrosis transmembrane conductance regulator by vasoactive intestinal peptide in the human nasal epithelial cell line JME/CF15.
Cystic Fibrosis
Rescue of multiple class II CFTR mutations by elexacaftor+tezacaftor+ivacaftor mediated in part by the dual activities of elexacaftor as both corrector and potentiator.
Cystic Fibrosis
Rescuing cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by transcomplementation.
Cystic Fibrosis
Residual chloride secretion in intestinal tissue of deltaF508 homozygous twins and siblings with cystic fibrosis. The European CF Twin and Sibling Study Consortium.
Cystic Fibrosis
Respiratory epithelial gene expression in patients with mild and severe cystic fibrosis lung disease.
Cystic Fibrosis
Respiratory exacerbations in childhood associated with compound heterozygosity Phe508del/Arg117His-7T of the cystic fibrosis transmembrane regulator gene.
Cystic Fibrosis
Respiratory syncytial virus engineered to express the cystic fibrosis transmembrane conductance regulator corrects the bioelectric phenotype of human cystic fibrosis airway epithelium in vitro.
Cystic Fibrosis
Respiratory syncytial virus infection disrupts monolayer integrity and function in cystic fibrosis airway cells.
Cystic Fibrosis
Respiratory syncytial virus infection in a murine model of cystic fibrosis.
Cystic Fibrosis
Response to Acute Lung Infection with Mucoid Pseudomonas aeruginosa in Cystic Fibrosis Mice.
Cystic Fibrosis
Restoration of bacterial killing activity of human respiratory cystic fibrosis cells through cationic vector-mediated cystic fibrosis transmembrane conductance regulator gene transfer.
Cystic Fibrosis
Restoration of F508-del Function by Transcomplementation: The Partners Meet in the Endoplasmic Reticulum.
Cystic Fibrosis
Restoration of NBD1 thermal stability is necessary and sufficient to correct ?F508 CFTR folding and assembly.
Cystic Fibrosis
Restoration of the cystic fibrosis transmembrane conductance regulator function by splicing modulation.
Cystic Fibrosis
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation.
Cystic Fibrosis
Resveratrol ameliorates abnormalities of fluid and electrolyte secretion in a hypoxia-Induced model of acquired CFTR deficiency.
Cystic Fibrosis
Resveratrol and ivacaftor are additive G551D CFTR-channel potentiators: therapeutic implications for cystic fibrosis sinus disease.
Cystic Fibrosis
Resveratrol dimer trans-?-viniferin prevents rotaviral diarrhea in mice by inhibition of the intestinal calcium-activated chloride channel.
Cystic Fibrosis
Resveratrol enhances airway surface liquid depth in sinonasal epithelium by increasing cystic fibrosis transmembrane conductance regulator open probability.
Cystic Fibrosis
Resveratrol rescues cAMP-dependent anionic transport in the cystic fibrosis pancreatic cell line CFPAC1.
Cystic Fibrosis
Reticulocyte lysate as a model system to study endoplasmic reticulum membrane protein degradation.
Cystic Fibrosis
Retinoic acid promotes stem cell differentiation and embryonic development by transcriptionally activating CFTR.
Cystic Fibrosis
Retrospective study of the cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Guthrie cards from a large cohort of neonatal screening for cystic fibrosis.
Cystic Fibrosis
Reversal of cystic fibrosis phenotype in a cultured Delta508 cystic fibrosis transmembrane conductance regulator cell line by oligonucleotide insertion.
Cystic Fibrosis
Reverse transcription-competitive multiplex PCR improves quantification of mRNA in clinical samples--application to the low abundance CFTR mRNA.
Cystic Fibrosis
Reverse transcription-polymerase chain reaction (RT-PCR) phenotypic analysis of cell cultures of human tracheal epithelium, tracheobronchial glands, and lung carcinomas.
Cystic Fibrosis
Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR.
Cystic Fibrosis
Review. ATP hydrolysis-driven gating in cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Revisiting CFTR inhibition: a comparative study of CFTRinh -172 and GlyH-101 inhibitors.
Cystic Fibrosis
Revisiting cystic fibrosis transmembrane conductance regulator structure and function.
Cystic Fibrosis
Revisiting the Role of CFTR and Counterion Permeability in the pH Regulation of Endocytic Organelles.
Cystic Fibrosis
ReXSpecies--a tool for the analysis of the evolution of gene regulation across species.
Cystic Fibrosis
Rheological effects of hypertonic saline and sodium bicarbonate solutions on cystic fibrosis sputum in vitro.
Cystic Fibrosis
Rhesus ?-defensin-1 (RTD-1) exhibits in vitro and in vivo activity against cystic fibrosis strains of Pseudomonas aeruginosa.
Cystic Fibrosis
Right-angle light scattering to assay basal and regulated plasma membrane Cl- conductances.
Cystic Fibrosis
Riociguat for the treatment of Phe508del homozygous adults with cystic fibrosis.
Cystic Fibrosis
Rme-1 regulates the recycling of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
RNA Interference Screen to Identify Kinases That Suppress Rescue of ?F508-CFTR.
Cystic Fibrosis
RNF185 is a novel E3 ligase of Endoplasmic Reticulum Associated Degradation (ERAD) that targets Cystic Fibrosis Transmembrane conductance Regulator (CFTR).
Cystic Fibrosis
Robust method for TALEN-edited correction of pF508del in patient-specific induced pluripotent stem cells.
Cystic Fibrosis
Robust Stimulation of W1282X-CFTR Channel Activity by a Combination of Allosteric Modulators.
Cystic Fibrosis
Roflumilast combined with adenosine increases mucosal hydration in human airway epithelial cultures after cigarette smoke exposure.
Cystic Fibrosis
Roflumilast reverses CFTR-mediated ion transport dysfunction in cigarette smoke-exposed mice.
Cystic Fibrosis
Role for cystic fibrosis transmembrane conductance regulator protein in a glutathione response to bronchopulmonary pseudomonas infection.
Cystic Fibrosis
Role for PKC alpha and PKC epsilon in down-regulation of CFTR mRNA in a human epithelial liver cell line.
Cystic Fibrosis
Role of ATP binding and hydrolysis in the gating of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Role of Binding and Nucleoside Diphosphate Kinase A in the Regulation of the Cystic Fibrosis Transmembrane Conductance Regulator by AMP-Activated Protein Kinase.
Cystic Fibrosis
Role of CFTR expressed by neutrophils in modulating acute lung inflammation and injury in mice.
Cystic Fibrosis
Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice.
Cystic Fibrosis
Role of CFTR in oxidative stress and suicidal death of renal cells during cisplatin-induced nephrotoxicity.
Cystic Fibrosis
Role of CFTR mutation analysis in the diagnostic algorithm for cystic fibrosis.
Cystic Fibrosis
Role of CFTR's intrinsic adenylate kinase activity in gating of the Cl(-) channel.
Cystic Fibrosis
Role of CFTR's PDZ1-binding domain, NBF1 and Cl(-) conductance in inhibition of epithelial Na(+) channels in Xenopus oocytes.
Cystic Fibrosis
Role of chloride transport proteins in the vasorelaxant action of nitroprusside in isolated rat aorta.
Cystic Fibrosis
Role of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in patients with chronic sinopulmonary disease.
Cystic Fibrosis
Role of cystic fibrosis transmembrane conductance regulator in pulmonary clearance of Pseudomonas aeruginosa in vivo.
Cystic Fibrosis
Role of down-regulated in adenoma anion exchanger in HCO3- secretion across murine duodenum.
Cystic Fibrosis
Role of epithelial sodium channels in the regulation of lung fluid homeostasis.
Cystic Fibrosis
Role of Hsc70 binding cycle in CFTR folding and endoplasmic reticulum-associated degradation.
Cystic Fibrosis
Role of IKK and ERK pathways in intrinsic inflammation of cystic fibrosis airways.
Cystic Fibrosis
Role of IL-10 deficiency in excessive nuclear factor-kappaB activation and lung inflammation in cystic fibrosis transmembrane conductance regulator knockout mice.
Cystic Fibrosis
Role of individual R domain phosphorylation sites in CFTR regulation by protein kinase A.
Cystic Fibrosis
Role of inflammation and oxidative stress in tissue damage associated with cystic fibrosis: CAPE as a future therapeutic strategy.
Cystic Fibrosis
Role of Interaction and Nucleoside Diphosphate Kinase B in Regulation of the Cystic Fibrosis Transmembrane Conductance Regulator Function by cAMP-Dependent Protein Kinase A.
Cystic Fibrosis
Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections.
Cystic Fibrosis
Role of NHERF1, cystic fibrosis transmembrane conductance regulator, and cAMP in the regulation of aquaporin 9.
Cystic Fibrosis
Role of pertussis toxin-sensitive G-proteins in intracellular Ca2+ release and apoptosis induced by inhibiting cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channels in HepG2 human hepatoblastoma cells.
Cystic Fibrosis
Role of phosphodiesterase-4 inhibitors in chronic obstructive pulmonary disease.
Cystic Fibrosis
Role of pirfenidone in TGF-? pathways and other inflammatory pathways in acute respiratory syndrome coronavirus 2 (SARS-Cov-2) infection: a theoretical perspective.
Cystic Fibrosis
Role of Protein Kinase A-Mediated Phosphorylation in CFTR Channel Activity Regulation.
Cystic Fibrosis
Role of protein phosphatases in the activation of CFTR (ABCC7) by genistein and bromotetramisole.
Cystic Fibrosis
Role of Smad3 and p38 Signalling in Cigarette Smoke-induced CFTR and BK dysfunction in Primary Human Bronchial Airway Epithelial Cells.
Cystic Fibrosis
Role of sphingosine-1-phosphate phosphohydrolase 1 in the regulation of resistance artery tone.
Cystic Fibrosis
Role of the actin cytoskeleton in the regulation of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Role of the amiloride-sensitive epithelial Na+ channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease.
Cystic Fibrosis
Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections.
Cystic Fibrosis
Role of the cystic fibrosis transmembrane conductance regulator in internalization of Pseudomonas aeruginosa by polarized respiratory epithelial cells.
Cystic Fibrosis
Role of the energy sensor AMP-activated protein kinase in renal physiology and disease.
Cystic Fibrosis
Role of the extracellular loop in the folding of a CFTR transmembrane helical hairpin.
Cystic Fibrosis
Role of the juxtamembrane region of cytoplasmic loop 3 in the gating and conductance of the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Role of the PDZ scaffolding protein in tubule cells in maintenance of polarised function.
Cystic Fibrosis
Role of tyrosine phosphorylation in the muscarinic activation of the cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
Roles of volume-regulatory anion channels, VSOR and Maxi-Cl, in apoptosis, cisplatin resistance, necrosis, ischemic cell death, stroke and myocardial infarction.
Cystic Fibrosis
RPTOR, a novel target of miR-155, elicits a fibrotic phenotype of cystic fibrosis lung epithelium by upregulating CTGF.
Cystic Fibrosis
S-carbocysteine-lysine salt monohydrate and cAMP cause non-additive activation of the cystic fibrosis transmembrane regulator channel in human respiratory epithelium.
Cystic Fibrosis
S-CMC-Lys-dependent stimulation of electrogenic glutathione secretion by human respiratory epithelium.
Cystic Fibrosis
S-nitrosoglutathione increases cystic fibrosis transmembrane regulator maturation.
Cystic Fibrosis
S-Nitrosothiols increases cystic fibrosis transmembrane regulator expression and maturation in the cell surface.
Cystic Fibrosis
S-nitrosylating agents: a novel class of compounds that increase cystic fibrosis transmembrane conductance regulator expression and maturation in epithelial cells.
Cystic Fibrosis
S737F is a new CFTR mutation typical of patients originally from the Tuscany region in Italy.
Cystic Fibrosis
Saccharomyces cerivisiae as a model system for kidney disease: what can yeast tell us about renal function?
Cystic Fibrosis
Safety and biological efficacy of an adeno-associated virus vector-cystic fibrosis transmembrane regulator (AAV-CFTR) in the cystic fibrosis maxillary sinus.
Cystic Fibrosis
Safety and efficacy of the cystic fibrosis transmembrane conductance regulator potentiator icenticaftor (QBW251).
Cystic Fibrosis
Safety and efficacy of treatment with lumacaftor in combination with ivacaftor in younger patients with cystic fibrosis.
Cystic Fibrosis
Safety of adenovirus-mediated transfer of the human cystic fibrosis transmembrane conductance regulator cDNA to the lungs of nonhuman primates.
Cystic Fibrosis
Safety of aerosolized INS 365 in patients with mild to moderate cystic fibrosis: results of a phase I multi-center study.
Cystic Fibrosis
Safety of airway gene transfer with Ad2/CFTR2: aerosol administration in the nonhuman primate.
Cystic Fibrosis
Safety of local delivery of low- and intermediate-dose adenovirus gene transfer vectors to individuals with a spectrum of morbid conditions.
Cystic Fibrosis
Safety of single-dose administration of an adeno-associated virus (AAV)-CFTR vector in the primate lung.
Cystic Fibrosis
Salinity-dependent expression of ncc2 in opercular epithelium and gill of mummichog (Fundulus heteroclitus).
Cystic Fibrosis
Salivary Biomarkers and Oral Microbial Load in Relation to the Dental Status of Adults with Cystic Fibrosis.
Cystic Fibrosis
Salivary electrolyte concentrations are associated with cystic fibrosis transmembrane regulator genotypes.
Cystic Fibrosis
Salivary Thiocyanate as a Biomarker of Cystic Fibrosis Transmembrane Regulator Function.
Cystic Fibrosis
Salmeterol Restores Secretory Functions in Cystic Fibrosis Airway Submucosal Gland Serous Cells.
Cystic Fibrosis
Salmonella enterica serovar typhi modulates cell surface expression of its receptor, the cystic fibrosis transmembrane conductance regulator, on the intestinal epithelium.
Cystic Fibrosis
Salt stress increases abundance and glycosylation of CFTR localized at apical surfaces of salt gland secretory cells.
Cystic Fibrosis
SARS-CoV-2 may hijack GPCR signaling pathways to dysregulate lung ion and fluid transport.
Cystic Fibrosis
Scanning the cystic fibrosis transmembrane conductance regulator gene using high-resolution DNA melting analysis.
Cystic Fibrosis
Screen-Printed Sensor for Low-Cost Chloride Analysis in Sweat for Rapid Diagnosis and Monitoring of Cystic Fibrosis.
Cystic Fibrosis
Screening for cystic fibrosis in newborn infants: results of a pilot programme based on a two tier protocol (IRT/DNA/IRT) in the Italian population.
Cystic Fibrosis
Screening for cystic fibrosis transmembrane conductance regulator gene mutations in men included in an intracytoplasmic sperm injection programme.
Cystic Fibrosis
Screening for F508del as a first step in the molecular diagnosis of cystic fibrosis.
Cystic Fibrosis
Screening for mutations in the cystic fibrosis transmembrane regulator gene in an infertility clinic.
Cystic Fibrosis
Screening for non-delta F508 mutations in five exons of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in Italy.
Cystic Fibrosis
Screening for regulatory variants in 460kb encompassing the CFTR locus in cystic fibrosis patients.
Cystic Fibrosis
Screening methods for cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in non-human primates.
Cystic Fibrosis
Screening of ?F508 mutation and IVS8-poly T polymorphism in CFTR gene in Tunisian infertile men without CBAVD.
Cystic Fibrosis
Search for mutations in pancreatic sufficient cystic fibrosis Italian patients: detection of 90% of molecular defects and identification of three novel mutations.
Cystic Fibrosis
Sec16 in Conventional and Unconventional Exocytosis: Working at the Interface of Membrane Traffic and Secretory Autophagy?
Cystic Fibrosis
Secretory cells of the airway express molecules of the chemoreceptive cascade.
Cystic Fibrosis
Secretory low-molecular-weight phospholipases A2 and their specific receptor in bile ducts of patients with intrahepatic calculi: factors of chronic proliferative cholangitis.
Cystic Fibrosis
Segmental heterogeneity of electrogenic secretions in human ascending colon and rectum.
Cystic Fibrosis
Selective activation of cystic fibrosis transmembrane conductance regulator Cl- and HCO3- conductances.
Cystic Fibrosis
Selective Binding of HSC70 and its Co-Chaperones to Structural Hotspots on CFTR.
Cystic Fibrosis
Selective block of swelling-activated Cl- channels over cAMP-dependent Cl- channels in ventricular myocytes.
Cystic Fibrosis
Self-assembled peptide-poloxamine nanoparticles enable in vitro and in vivo genome restoration for cystic fibrosis.
Cystic Fibrosis
Self-renewal and differentiation of rat Epididymal basal cells using a novel in vitro organoid model.
Cystic Fibrosis
Seminal plasma characteristics as indicators of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in men with obstructive azoospermia.
Cystic Fibrosis
Sensitivity of a renal K+ channel (ROMK2) to the inhibitory sulfonylurea compound glibenclamide is enhanced by coexpression with the ATP-binding cassette transporter cystic fibrosis transmembrane regulator.
Cystic Fibrosis
Sequence analyses of presenilin mutations linked to familial Alzheimer's disease.
Cystic Fibrosis
Sequence analysis of the CEN12 region of Saccharomyces cerevisiae on a 43.7 kb fragment of chromosome XII including an open reading frame homologous to the human cystic fibrosis transmembrane conductance regulator protein CFTR.
Cystic Fibrosis
Sequence analysis of the cystic fibrosis gene in patients with disseminated bronchiectatic lung disease. Application in the identification of a cystic fibrosis patient with atypical clinical course.
Cystic Fibrosis
Sequence and structural homology among membrane-associated domains of CFTR and certain transporter proteins.
Cystic Fibrosis
Sequence homologies between nucleotide binding regions of CFTR and G-proteins suggest structural and functional similarities.
Cystic Fibrosis
Sequence Hydropathy Dominates Membrane Protein Response to Detergent Solubilization.
Cystic Fibrosis
Sequence specific generation of a DNA panhandle permits PCR amplification of unknown flanking DNA.
Cystic Fibrosis
Sequence-specific retention and regulated integration of a nascent membrane protein by the endoplasmic reticulum Sec61 translocon.
Cystic Fibrosis
Sequences within the coding regions of clotting factor VIII and CFTR block transcriptional elongation.
Cystic Fibrosis
Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
SERCA Pump Inhibitors Do Not Correct Biosynthetic Arrest of {Delta}F508 CFTR in Cystic Fibrosis.
Cystic Fibrosis
Sertoli cell expression of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Serum and glucocorticoid-inducible kinase1 increases plasma membrane wt-CFTR in human airway epithelial cells by inhibiting its endocytic retrieval.
Cystic Fibrosis
Serum inflammatory profiles in cystic fibrosis mice with and without Bordetella pseudohinzii infection.
Cystic Fibrosis
Serum- and glucocorticoid-induced protein kinase 1 (SGK1) increases the cystic fibrosis transmembrane conductance regulator (CFTR) in airway epithelial cells by phosphorylating Shank2E protein.
Cystic Fibrosis
Severe allergic bronchopulmonary aspergillosis in an infant with cystic fibrosis and her asthmatic father.
Cystic Fibrosis
Severe cystic fibrosis associated with a deltaF508/R347H + D979A compound heterozygous genotype.
Cystic Fibrosis
Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis.
Cystic Fibrosis
Severed channels probe regulation of gating of cystic fibrosis transmembrane conductance regulator by its cytoplasmic domains.
Cystic Fibrosis
Severed molecules functionally define the boundaries of the cystic fibrosis transmembrane conductance regulator's NH(2)-terminal nucleotide binding domain.
Cystic Fibrosis
Sexual maturation and changes in water and salt transport components in the kidney and intestine of three-spined stickleback (Gasterosteus aculeatus L.).
Cystic Fibrosis
SFPQ rescues F508del-CFTR expression and function in cystic fibrosis bronchial epithelial cells.
Cystic Fibrosis
Short communication: novel truncating mutations in the CFTR gene causing a severe form of cystic fibrosis in Italian patients.
Cystic Fibrosis
Short- and long-term low-salinity acclimation effects on the branchial and intestinal gene expression in the European seabass (Dicentrarchus labrax).
Cystic Fibrosis
Short-chain fatty acids affect cystic fibrosis airway inflammation and bacterial growth.
Cystic Fibrosis
Short-term consequences of F508del-CFTR thermal instability on CFTR-dependent transepithelial currents in human airway epithelial cells.
Cystic Fibrosis
Short-term low-salinity tolerance by the longhorn sculpin, Myoxocephalus octodecimspinosus.
Cystic Fibrosis
Shrinkage activates a nonselective conductance: involvement of a Walker-motif protein and PKC.
Cystic Fibrosis
Signaling Cascade Involved in Rapid Stimulation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Dexamethasone.
Cystic Fibrosis
Signature motifs identify an acinetobacter cif virulence factor with epoxide hydrolase activity.
Cystic Fibrosis
Silencing of the Hsp70-specific nucleotide-exchange factor BAG3 corrects the F508del-CFTR variant by restoring autophagy.
Cystic Fibrosis
Simple binding of protein kinase A prior to phosphorylation allows CFTR anion channels to be opened by nucleotides.
Cystic Fibrosis
Simple method for haplotyping the poly(TG) repeat in individuals carrying the IVS8 5T allele in the CFTR gene.
Cystic Fibrosis
Simple multiplex genotyping by surface-enhanced resonance Raman scattering.
Cystic Fibrosis
Simulation analysis of intracellular Na+ and Cl- homeostasis during beta 1-adrenergic stimulation of cardiac myocyte.
Cystic Fibrosis
Simulation of Cl(-) Secretion in Epithelial Tissues: New Methodology Estimating Activity of Electro-Neutral Cl(-) Transporter.
Cystic Fibrosis
Simultaneous cycle sequencing assessment of (TG)m and Tn tract length in CFTR gene.
Cystic Fibrosis
Simultaneous screening for 11 mutations in the cystic fibrosis transmembrane conductance regulator gene by multiplex amplification and reverse dot-blot.
Cystic Fibrosis
Single intragenic microsatellite preimplantation genetic diagnosis for cystic fibrosis provides positive allele identification of all CFTR genotypes for informative couples.
Cystic Fibrosis
Single-cell-resolved differentiation of human induced pluripotent stem cells into pancreatic duct-like organoids on a microwell chip.
Cystic Fibrosis
Single-particle tracking of membrane protein diffusion in a potential: simulation, detection, and application to confined diffusion of CFTR Cl- channels.
Cystic Fibrosis
Single-strand conformation polymorphism (SSCP) analysis of exon 11 of the CFTR gene reliably detects more than one third of non-delta F508 mutations in German cystic fibrosis patients.
Cystic Fibrosis
Single-stranded conformation polymorphism analysis of the CFTR gene in Slovenian cystic fibrosis patients: detection of mutations and sequence variations.
Cystic Fibrosis
Sinus hypoplasia in the cystic fibrosis rat resolves in the absence of chronic infection.
Cystic Fibrosis
Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis.
Cystic Fibrosis
Sites associated with Kalydeco binding on human Cystic Fibrosis Transmembrane Conductance Regulator revealed by Hydrogen/Deuterium Exchange.
Cystic Fibrosis
Sixty-five years since the New York heat wave: advances in sweat testing for cystic fibrosis.
Cystic Fibrosis
Skeletal muscle contractility and fatigability in adults with cystic fibrosis.
Cystic Fibrosis
Skin ionocyte remodeling in the amphibious mangrove rivulus fish (Kryptolebias marmoratus).
Cystic Fibrosis
Skipping of exon 12 as a consequence of a point mutation (1898 + 5G-->T) in the cystic fibrosis transmembrane conductance regulator gene found in a consanguineous Chinese family.
Cystic Fibrosis
SLC26 anion exchangers in uterine epithelial cells and spermatozoa: clues from the past and hints to the future.
Cystic Fibrosis
SLC26 anion exchangers of guinea pig pancreatic duct: molecular cloning and functional characterization.
Cystic Fibrosis
Slc26a11 is prominently expressed in the brain and functions as a chloride channel: expression in Purkinje cells and stimulation of V H(+)-ATPase.
Cystic Fibrosis
Slc26a3 deficiency is associated with epididymis dysplasia and impaired sperm fertilization potential in the mouse.
Cystic Fibrosis
Slc26a9 is inhibited by the R-region of the cystic fibrosis transmembrane conductance regulator via the STAS domain.
Cystic Fibrosis
SLC9A3 Affects Vas Deferens Development and Associates with Taiwanese Congenital Bilateral Absence of the Vas Deferens.
Cystic Fibrosis
Sleep-Related Rhythmic Movement Disorder in Triplets: Evidence for Genetic Predisposition?
Cystic Fibrosis
Slow conversions among subconductance states of cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Small changes in gene expression of targeted osmoregulatory genes when exposing marine and freshwater threespine stickleback (Gasterosteus aculeatus) to abrupt salinity transfers.
Cystic Fibrosis
Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway.
Cystic Fibrosis
Small heat-shock proteins select deltaF508-CFTR for endoplasmic reticulum-associated degradation.
Cystic Fibrosis
Small Hsps as Therapeutic Targets of Cystic Fibrosis Transmembrane Conductance Regulator Protein.
Cystic Fibrosis
Small interfering peptide (siP) for in vivo examination of the developing lung interactonome.
Cystic Fibrosis
Small Molecule Anion Carriers Correct Abnormal Airway Surface Liquid Properties in Cystic Fibrosis Airway Epithelia.
Cystic Fibrosis
Small-molecule CFTR activators increase tear secretion and prevent experimental dry eye disease.
Cystic Fibrosis
Small-molecule CFTR inhibitors slow cyst growth in polycystic kidney disease.
Cystic Fibrosis
Small-molecule inhibitors of the cystic fibrosis transmembrane conductance regulator increase pancreatic endocrine cell development in rat and mouse.
Cystic Fibrosis
Small-molecule ion channels increase host defences in cystic fibrosis airway epithelia.
Cystic Fibrosis
Small-molecule screen identifies inhibitors of a human intestinal calcium-activated chloride channel.
Cystic Fibrosis
Small-molecule vasopressin-2 receptor antagonist identified by a g-protein coupled receptor "pathway" screen.
Cystic Fibrosis
Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation.
Cystic Fibrosis
Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR.
Cystic Fibrosis
Spatiotemporal coupling of cAMP transporter to CFTR chloride channel function in the gut epithelia.
Cystic Fibrosis
Specialized Pro-resolving Mediators Regulate Alveolar Fluid Clearance during Acute Respiratory Distress Syndrome.
Cystic Fibrosis
Species-specific differences in mouse and human airway epithelial biology of recombinant adeno-associated virus transduction.
Cystic Fibrosis
Specific rescue of cystic fibrosis transmembrane conductance regulator processing mutants using pharmacological chaperones.
Cystic Fibrosis
Specific resistance to Pseudomonas aeruginosa infection in zebrafish is mediated by the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Specificity of NHERF1 regulation of GPCR signaling and function in human airway smooth muscle.
Cystic Fibrosis
Spectrum and distribution of CFTR gene mutations in asthma and chronic pancreatitis cases of North Indian population.
Cystic Fibrosis
Spectrum of CFTR mutations in Chechen cystic fibrosis patients: high frequency of c.1545_1546delTA (p.Tyr515X; 1677delTA) and c.274G>A (p.Glu92Lys, E92K) mutations in North Caucasus.
Cystic Fibrosis
Spectrum of CFTR mutations in Mexican cystic fibrosis patients: identification of five novel mutations (W1098C, 846delT, P750L, 4160insGGGG and 297-1G-->A).
Cystic Fibrosis
Spectrum of cystic fibrosis mutations in Serbia and Montenegro and strategy for prenatal diagnosis.
Cystic Fibrosis
Spectrum of ion channels in alveolar epithelial cells: implications for alveolar fluid balance.
Cystic Fibrosis
Spectrum of mutations and variants/haplotypes of CFTR and genotype-phenotype correlation in idiopathic chronic pancreatitis and controls in Chinese by complete analysis.
Cystic Fibrosis
Speeding Up the Identification of Cystic Fibrosis Transmembrane Conductance Regulator-Targeted Drugs: An Approach Based on Bioinformatics Strategies and Surface Plasmon Resonance.
Cystic Fibrosis
Sperm cystic fibrosis transmembrane conductance regulator expression level is relevant to fecundity of healthy couples.
Cystic Fibrosis
Sperm retrieval and intracytoplasmic sperm injection outcomes in men with cystic fibrosis disease versus congenital bilateral absence of the vas deferens.
Cystic Fibrosis
Sphingolipids and plasma membrane hydrolases in human primary bronchial cells during differentiation and their altered patterns in cystic fibrosis.
Cystic Fibrosis
Sphingomyelinase decreases transepithelial anion secretion in airway epithelial cells in part by inhibiting CFTR-mediated apical conductance.
Cystic Fibrosis
Sphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity.
Cystic Fibrosis
Sphingosine-1-Phosphate Signaling Regulates Myogenic Responsiveness in Human Resistance Arteries.
Cystic Fibrosis
SPI-0211 activates T84 cell chloride transport and recombinant human ClC-2 chloride currents.
Cystic Fibrosis
Spiperone, identified through compound screening, activates calcium-dependent chloride secretion in the airway.
Cystic Fibrosis
Spliceosome-Mediated RNA Trans-Splicing with Recombinant Adeno-Associated Virus Partially Restores Cystic Fibrosis Transmembrane Conductance Regulator Function to Polarized Human Cystic Fibrosis Airway Epithelial Cells.
Cystic Fibrosis
Spliceosome-mediated RNA trans-splicing with recombinant adeno-associated virus partially restores cystic fibrosis transmembrane conductance regulator function to polarized human cystic fibrosis airway epithelial cells.
Cystic Fibrosis
Splicing defects in the CFTR gene: Minigene analysis of two mutations, 1811+1G>C and 1898+3A>G.
Cystic Fibrosis
Splicing factors induce cystic fibrosis transmembrane regulator exon 9 skipping through a nonevolutionary conserved intronic element.
Cystic Fibrosis
SPLUNC1 degradation by the cystic fibrosis mucosal environment drives airway surface liquid dehydration.
Cystic Fibrosis
SPX-101 Is a Novel Epithelial Sodium Channel-targeted Therapeutic for Cystic Fibrosis That Restores Mucus Transport.
Cystic Fibrosis
Src signaling links mediators of inflammation to Cx43 gap junction channels in primary and transformed CFTR-expressing airway cells.
Cystic Fibrosis
STa and cGMP stimulate CFTR translocation to the surface of villus enterocytes in rat jejunum and is regulated by protein kinase G.
Cystic Fibrosis
Stability Prediction for Mutations in the Cytosolic Domains of Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
Stabilization of a nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator yields insight into disease-causing mutations.
Cystic Fibrosis
Stable ATP binding mediated by a partial NBD dimer of the CFTR chloride channel.
Cystic Fibrosis
Stable in vivo expression of the cystic fibrosis transmembrane conductance regulator with an adeno-associated virus vector.
Cystic Fibrosis
Standardized Measurement of Nasal Membrane Transepithelial Potential Difference (NPD).
Cystic Fibrosis
State-dependent access of anions to the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Cystic Fibrosis
State-dependent blocker interactions with the CFTR chloride channel: implications for gating the pore.
Cystic Fibrosis
State-dependent chemical reactivity of an engineered cysteine reveals conformational changes in the outer vestibule of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
State-dependent inhibition of cystic fibrosis transmembrane conductance regulator chloride channels by a novel peptide toxin.
Cystic Fibrosis
Stem cell-derived organoids to model gastrointestinal facets of cystic fibrosis.
Cystic Fibrosis
Steviol Reduces MDCK Cyst Formation and Growth by Inhibiting CFTR Channel Activity and Promoting Proteasome-Mediated CFTR Degradation.
Cystic Fibrosis
Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease.
Cystic Fibrosis
Stimulation by alkylxanthines of chloride efflux in CFPAC-1 cells does not involve A1 adenosine receptors.
Cystic Fibrosis
Stimulation by caveolin-1 of the hypotonicity-induced release of taurine and ATP at basolateral, but not apical, membrane of Caco-2 cells.
Cystic Fibrosis
Stimulation effect of wide type CFTR chloride channel by the naturally occurring flavonoid tangeretin.
Cystic Fibrosis
Stimulation of alveolar epithelial fluid clearance in human lungs by exogenous epinephrine.
Cystic Fibrosis
Stimulation of beta 2-adrenergic receptor increases cystic fibrosis transmembrane conductance regulator expression in human airway epithelial cells through a cAMP/protein kinase A-independent pathway.
Cystic Fibrosis
Stimulation of chloride secretion by baicalein in isolated rat distal colon.
Cystic Fibrosis
Stimulation of cystic fibrosis transmembrane conductance regulator-dependent short-circuit currents across DeltaF508 murine intestines.
Cystic Fibrosis
Stimulation of epithelial sodium channel activity by the sulfonylurea glibenclamide.
Cystic Fibrosis
Stimulation of Intestinal Cl- Secretion Through CFTR by Caffeine Intake in Salt-Sensitive Hypertensive Rats.
Cystic Fibrosis
Stimulation of nuclear receptor peroxisome proliferator-activated receptor-? limits NF-?B-dependent inflammation in mouse cystic fibrosis biliary epithelium.
Cystic Fibrosis
Stimulation of rat erythrocyte P2X7 receptor induces the release of epoxyeicosatrienoic acids.
Cystic Fibrosis
Stimulation of the cystic fibrosis transmembrane regulator expression by estrogen in vivo.
Cystic Fibrosis
Stimulation of Wild-Type, F508del- and G551D-CFTR Chloride Channels by Non-Toxic Modified pyrrolo[2,3-b]pyrazine Derivatives.
Cystic Fibrosis
Stimulatory and inhibitory protein kinase C consensus sequences regulate the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Stoichiometry and novel gating mechanism within the cystic fibrosis transmembrane conductance regulator channel.
Cystic Fibrosis
Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro.
Cystic Fibrosis
Strain-dependent pulmonary gene expression profiles of a cystic fibrosis mouse model.
Cystic Fibrosis
Strain-specific variants of the mouse Cftr promoter region reveal transcriptional regulatory elements.
Cystic Fibrosis
Strategies against Nonsense: Oxadiazoles as Translational Readthrough-Inducing Drugs (TRIDs).
Cystic Fibrosis
Strategies for cystic fibrosis transmembrane conductance regulator inhibition: from molecular mechanisms to treatment for secretory diarrhoeas.
Cystic Fibrosis
Strategies for Unambiguous Detection of Allelic Heterozygosity via Direct DNA Sequencing of PCR Products: Application to the HLA DRB1 Locus.
Cystic Fibrosis
Stratifying infants with cystic fibrosis for disease severity using intestinal organoid swelling as a biomarker of CFTR function.
Cystic Fibrosis
Streptomycin treatment alters the intestinal microbiome, pulmonary T cell profile and airway hyperresponsiveness in a cystic fibrosis mouse model.
Cystic Fibrosis
Strong toll-like receptor responses in cystic fibrosis patients are associated with higher lung function.
Cystic Fibrosis
Structural analog of sildenafil identified as a novel corrector of the F508del-CFTR trafficking defect.
Cystic Fibrosis
Structural analogues of roscovitine rescue the intracellular traffic and the function of ER-retained ABCB4 variants in cell models.
Cystic Fibrosis
Structural analysis of cloned plasma membrane proteins by freeze-fracture electron microscopy.
Cystic Fibrosis
Structural and ionic determinants of 5-nitro-2-(3-phenylprophyl-amino)-benzoic acid block of the CFTR chloride channel.
Cystic Fibrosis
Structural basis for misfolding at a disease phenotypic position in CFTR: Comparison of TM3/4 helix-loop-helix constructs with TM4 peptides.
Cystic Fibrosis
Structural basis for specificity and potency of xanthine derivatives as activators of the CFTR chloride channel.
Cystic Fibrosis
Structural basis for the channel function of a degraded ABC transporter, CFTR (ABCC7).
Cystic Fibrosis
Structural basis of the Na+/H+ exchanger regulatory factor PDZ1 interaction with the carboxyl-terminal region of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Structural basis of typhoid: Salmonella typhi type IVb pilin (PilS) and cystic fibrosis transmembrane conductance regulator interaction.
Cystic Fibrosis
Structural biology of Rad50 ATPase: ATP-driven conformational control in DNA double-strand break repair and the ABC-ATPase superfamily.
Cystic Fibrosis
Structural Changes Fundamental to Gating of the Cystic Fibrosis Transmembrane Conductance Regulator Anion Channel Pore.
Cystic Fibrosis
Structural changes of CFTR R region upon phosphorylation: a plastic platform for intramolecular and intermolecular interactions.
Cystic Fibrosis
Structural cues involved in endoplasmic reticulum degradation of G85E and G91R mutant cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Structural determinants for activation and block of CFTR-mediated chloride currents by apigenin.
Cystic Fibrosis
Structural effects of extracellular loop mutations in CFTR helical hairpins.
Cystic Fibrosis
Structural features of Escherichia coli heat-stable enterotoxin that activates membrane-associated guanylyl cyclase.
Cystic Fibrosis
Structural mechanisms for defective CFTR gating caused by the Q1412X mutation, a severe Class VI pathogenic mutation in cystic fibrosis.
Cystic Fibrosis
Structural models of CFTR-AMPK and CFTR-PKA interactions: R-domain flexibility is a key factor in CFTR regulation.
Cystic Fibrosis
Structural stability of purified human CFTR is systematically improved by mutations in nucleotide binding domain 1.
Cystic Fibrosis
Structure and dynamics of NBD1 from CFTR characterized using crystallography and hydrogen/deuterium exchange mass spectrometry.
Cystic Fibrosis
Structure and expression of the messenger RNA encoding the murine multidrug resistance protein, an ATP-binding cassette transporter.
Cystic Fibrosis
Structure and function of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Structure and in vitro substrate specificity of the murine multidrug resistance-associated protein.
Cystic Fibrosis
Structure and regulation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in killifish: A comparative genomics approach.
Cystic Fibrosis
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Structure of the human MDR3 gene and physical mapping of the human MDR locus.
Cystic Fibrosis
Structure-activity relationship of 1,4-dihydropyridines as potentiators of the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Structure-activity relationships of cyanoquinolines with corrector-potentiator activity in ?F508 cystic fibrosis transmembrane conductance regulator protein.
Cystic Fibrosis
Structure-function analysis of a double-mutant cystic fibrosis transmembrane conductance regulator protein occurring in disorders related to cystic fibrosis.
Cystic Fibrosis
Structure-guided combination therapy to potently improve the function of mutant CFTRs.
Cystic Fibrosis
Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant.
Cystic Fibrosis
Studies of the role for NSP4 in the pathogenesis of homologous murine rotavirus diarrhea.
Cystic Fibrosis
Studies on human porin. IX. Immunolocalization of porin and CFTR channels in human surface respiratory epithelium.
Cystic Fibrosis
Study of mutant and polyvariant mutant CFTR genes in patients with congenital absence of the vas deferens.
Cystic Fibrosis
Subcellular distribution of CFTR in rat intestine supports a physiologic role for CFTR regulation by vesicle traffic.
Cystic Fibrosis
Subepithelial fibrosis and degradation of the bronchial extracellular matrix in cystic fibrosis.
Cystic Fibrosis
Subject Review: Pancreatic Ductal Adenocarcinoma in the Setting of Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator Gene: Case Report and Review of the Literature.
Cystic Fibrosis
Submucosal gland distribution in the mouse has a genetic determination localized on chromosome 9.
Cystic Fibrosis
Substance P stimulates CFTR-dependent fluid secretion by mouse tracheal submucosal glands.
Cystic Fibrosis
Substitution of Yor1p NBD1 residues improves the thermal stability of Human Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
Substrates and inhibitors of human multidrug resistance associated proteins and the implications in drug development.
Cystic Fibrosis
Substrates of multidrug resistance-associated proteins block the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Successful targeting of the mouse cystic fibrosis transmembrane conductance regulator gene in embryonal stem cells.
Cystic Fibrosis
Suitability of oligonucleotide-mediated cystic fibrosis gene repair in airway epithelial cells.
Cystic Fibrosis
Sulfation of chondroitin/dermatan sulfate by cystic fibrosis pancreatic duct cells is not different from control cells.
Cystic Fibrosis
Sulfonylurea receptors inhibit the epithelial sodium channel (ENaC) by reducing surface expression.
Cystic Fibrosis
Sulfonylurea-sensitive K(+) transport is involved in Cl(-) secretion and cyst trowth by cultured ADPKD cells.
Cystic Fibrosis
SUMOylation represses the transcriptional activity of the Unfolded Protein Response transducer ATF6.
Cystic Fibrosis
Suppression of adenosine-activated chloride transport by ethanol in airway epithelia.
Cystic Fibrosis
Suppression of CFTR-mediated Cl Secretion of Airway Epithelium in Vitamin C-deficient Mice.
Cystic Fibrosis
Suppression of CFTR-mediated Cl(-) secretion by enhanced expression of epithelial Na(+) channels in mouse endometrial epithelium.
Cystic Fibrosis
Suppressive effects of 4-phenylbutyrate on the aggregation of Pael receptors and endoplasmic reticulum stress.
Cystic Fibrosis
Suppressor Mutations in the Transmembrane Segments of P-glycoprotein Promote Maturation of Processing Mutants and Disrupt a Subset of Drug-binding Sites.
Cystic Fibrosis
Surface expression of the cystic fibrosis transmembrane conductance regulator mutant DeltaF508 is markedly upregulated by combination treatment with sodium butyrate and low temperature.
Cystic Fibrosis
Survey of cystic fibrosis transmembrane conductance regulator genotypes in primary sclerosing cholangitis.
Cystic Fibrosis
Survey of patients with cystic fibrosis and caregivers decisions regarding CFTR modulators.
Cystic Fibrosis
Susceptibility to typhoid fever is associated with a polymorphism in the cystic fibrosis transmembrane conductance regulator (CFTR).
Cystic Fibrosis
Sustained Glycemic Control With Ivacaftor in Cystic Fibrosis-Related Diabetes.
Cystic Fibrosis
Sustained inhibition of IL-6 and IL-8 expression by ODN decoy to NF-?B delivered through respirable large porous particles in LPS-stimulated cystic fibrosis bronchial cells.
Cystic Fibrosis
Sweat chloride and immunoreactive trypsinogen in infants carrying two CFTR mutations and not affected by cystic fibrosis.
Cystic Fibrosis
Sweat Chloride Testing and Nasal Potential Difference (NPD) Are Primary Outcome Parameters in Treatment with Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators.
Cystic Fibrosis
Swelling-activated Ca2+ entry via TRPV4 channel is defective in cystic fibrosis airway epithelia.
Cystic Fibrosis
Swelling-activated, cystic fibrosis transmembrane conductance regulator-augmented ATP release and Cl- conductances in murine C127 cells.
Cystic Fibrosis
Swelling-induced Cl- current in guinea-pig atrial myocytes: inhibition by glibenclamide.
Cystic Fibrosis
Swelling-induced, CFTR-independent ATP release from a human epithelial cell line: lack of correlation with volume-sensitive cl(-) channels.
Cystic Fibrosis
Symmetric Snapback Primers for Scanning and Genotyping of the Cystic Fibrosis Transmembrane Conductance Regulator Gene.
Cystic Fibrosis
Synaptic scaffolding molecule binds to and regulates vasoactive intestinal polypeptide type-1 receptor in epithelial cells.
Cystic Fibrosis
Synergic action of insulin and genistein on Na+/K+/2Cl- cotransporter in renal epithelium.
Cystic Fibrosis
Synergistic activation of guinea-pig cardiac cystic fibrosis transmembrane conductance regulator by the tyrosine kinase inhibitor genistein and cAMP.
Cystic Fibrosis
Synergistic effects of cAMP- and calcium-mediated amylase secretion in isolated pancreatic acini from cystic fibrosis mice.
Cystic Fibrosis
Synergistic effects of cystic fibrosis transmembrane conductance regulator and aquaporin-9 in the rat epididymis.
Cystic Fibrosis
Synergistic effects of interferon gamma and tumour necrosis factor alpha on T84 cell function.
Cystic Fibrosis
Synergistic post-transcriptional regulation of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) by miR-101 and miR-494 specific binding.
Cystic Fibrosis
Synergistic Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Gating by Two Chemically Distinct Potentiators, Ivacaftor (VX-770) and 5-Nitro-2-(3-Phenylpropylamino) Benzoate.
Cystic Fibrosis
Synergy-based small-molecule screen using a human lung epithelial cell line yields ?F508-CFTR correctors that augment VX-809 maximal efficacy.
Cystic Fibrosis
Synonymous Codon Usage Affects the Expression of Wild Type and F508del CFTR.
Cystic Fibrosis
Synonymous mutations in CFTR exon 12 affect splicing and are not neutral in evolution.
Cystic Fibrosis
Syntaxin 16 binds to CFTR and regulates its membrane trafficking in epithelial cells.
Cystic Fibrosis
Syntaxin 3 is necessary for cAMP and cGMP-regulated exocytosis of CFTR: implications for enterotoxigenic diarrhea.
Cystic Fibrosis
Syntaxin 6 and CAL mediate the degradation of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Syntaxin 8 impairs trafficking of cystic fibrosis transmembrane conductance regulator (CFTR) and inhibits its channel activity.
Cystic Fibrosis
Synthesis and biological evaluation of thiazole derivatives on basic defects underlying cystic fibrosis.
Cystic Fibrosis
Synthesis and evaluation of esterified Hsp70 agonists in cellular models of protein aggregation and folding.
Cystic Fibrosis
Synthesis and properties of molecular probes for the rescue site on mutant cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Synthesis and structure-activity relationship of aminoarylthiazole derivatives as potential potentiators of the chloride transport defect in cystic fibrosis.
Cystic Fibrosis
Synthesis of pentasaccharides corresponding to the glycoform II of the outer core region of the Pseudomonas aeruginosa lipopolysaccharide.
Cystic Fibrosis
Synthesis, SAR, crystal structure, and biological evaluation of benzoquinoliziniums as activators of wild-type and mutant cystic fibrosis transmembrane conductance regulator channels.
Cystic Fibrosis
Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor.
Cystic Fibrosis
Systemic versus tissue-level prolactin signaling in a teleost during a tidal cycle.
Cystic Fibrosis
SYVN1, NEDD8, and FBXO2 Proteins Regulate ?F508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Ubiquitin-mediated Proteasomal Degradation.
Cystic Fibrosis
Tandem mass tag-based quantitative proteomic analysis of the liver reveals potential protein targets of Xiaochaihutang in CUMS model of depression.
Cystic Fibrosis
Tanshinone IIA sulfonate protects against cigarette smoke-induced COPD and down-regulation of CFTR in mice.
Cystic Fibrosis
Targeted Activation of Cystic Fibrosis Transmembrane Conductance Regulator.
Cystic Fibrosis
Targeted correction of a defective selectable marker gene in human epithelial cells by small DNA fragments.
Cystic Fibrosis
Targeted extended cystic fibrosis mutation testing on known and at-risk patients and relatives.
Cystic Fibrosis
Targeted Gene Next-Generation Sequencing in Chinese Children with Chronic Pancreatitis and Acute Recurrent Pancreatitis.
Cystic Fibrosis
Targeted inactivation of cystic fibrosis transmembrane conductance regulator chloride channel gene prevents ischemic preconditioning in isolated mouse heart.
Cystic Fibrosis
Targeted Next-Generation Sequencing Effectively Analyzed the Cystic Fibrosis Transmembrane Conductance Regulator Gene in Pancreatitis.
Cystic Fibrosis
Targeted proteomic quantitation of the absolute expression and turnover of cystic fibrosis transmembrane conductance regulator in the apical plasma membrane.
Cystic Fibrosis
Targeted quantitation of overexpressed and endogenous cystic fibrosis transmembrane conductance regulator using multiple reaction monitoring tandem mass spectrometry and oxygen stable isotope dilution.
Cystic Fibrosis
Targeted therapy for cystic fibrosis: cystic fibrosis transmembrane conductance regulator mutation-specific pharmacologic strategies.
Cystic Fibrosis
Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis.
Cystic Fibrosis
Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ?F508 cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Targeting CAL as a negative regulator of DeltaF508-CFTR cell-surface expression: an RNA interference and structure-based mutagenetic approach.
Cystic Fibrosis
Targeting cell-specific gene expression with an adenovirus vector containing the lacZ gene under the control of the CFTR promoter.
Cystic Fibrosis
Targeting cystic fibrosis inflammation in the age of CFTR modulators: focus on macrophages.
Cystic Fibrosis
Targeting different binding sites in the CFTR structures allows to synergistically potentiate channel activity.
Cystic Fibrosis
Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis.
Cystic Fibrosis
Targeting miRNA-based medicines to cystic fibrosis airway epithelial cells using nanotechnology.
Cystic Fibrosis
Targeting Molecular Chaperones for the Treatment of Cystic Fibrosis: Is It a Viable Approach?
Cystic Fibrosis
Targeting Nonsense: Optimization of 1,2,4-Oxadiazole TRIDs to Rescue CFTR Expression and Functionality in Cystic Fibrosis Cell Model Systems.
Cystic Fibrosis
Targeting of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Protein with a Technetium-99m Imaging Probe.
Cystic Fibrosis
Targeting the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Protein for the Treatment of Cystic Fibrosis.
Cystic Fibrosis
Targeting the Intracellular Environment in Cystic Fibrosis: Restoring Autophagy as a Novel Strategy to Circumvent the CFTR Defect.
Cystic Fibrosis
Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
TDP-43 binds heterogeneous nuclear ribonucleoprotein A/B through its C-terminal tail: an important region for the inhibition of cystic fibrosis transmembrane conductance regulator exon 9 splicing.
Cystic Fibrosis
Technical validation of a TM Biosciences Luminex-based multiplex assay for detecting the American College of Medical Genetics recommended cystic fibrosis mutation panel.
Cystic Fibrosis
Technological advances shed light on left ventricular cardiac disturbances in cystic fibrosis.
Cystic Fibrosis
Telomere shortening is associated with reduced duodenal HCOFormula secretory but normal gastric acid secretory capacity in aging mice.
Cystic Fibrosis
Temperature-dependent release of ATP from human erythrocytes: mechanism for the control of local tissue perfusion.
Cystic Fibrosis
Temporal regulation of CFTR expression during ovine lung development: implications for CF gene therapy.
Cystic Fibrosis
Temporal trends in healthcare resource use and associated costs of patients with cystic fibrosis.
Cystic Fibrosis
Terminal sialylation is altered in airway cells with impaired CFTR-mediated chloride transport.
Cystic Fibrosis
Testicular Cancer in a Lung Transplant Patient With Cystic Fibrosis: A Case Report.
Cystic Fibrosis
Testosterone decreases fluid and chloride secretions in the uterus of adult female rats via down-regulating cystic fibrosis transmembrane regulator (CFTR) expression and functional activity.
Cystic Fibrosis
Testosterone enhances expression and functional activity of epithelial sodium channel (ENaC), cystic fibrosis transmembrane regulator (CFTR) and sodium hydrogen exchanger (NHE) in vas deferens of sex-steroid deficient male rats.
Cystic Fibrosis
Testosterone regulates levels of cystic fibrosis transmembrane regulator, adenylate cyclase, and cAMP in the seminal vesicles of orchidectomized rats.
Cystic Fibrosis
Tetramethylpyrazine stimulates cystic fibrosis transmembrane conductance regulator-mediated anion secretion in distal colon of rodents.
Cystic Fibrosis
Tetrodotoxin-sensitive Na+ channels and muscarinic and purinergic receptors identified in human erythroid progenitor cells and red blood cell ghosts.
Cystic Fibrosis
Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del.
Cystic Fibrosis
Tezacaftor-ivacaftor use in routine care of adults with cystic fibrosis: a medicine use evaluation.
Cystic Fibrosis
Tezacaftor/Ivacaftor in Subjects with Cystic Fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR.
Cystic Fibrosis
TGF-?-induced IL-6 prevents development of acute lung injury in influenza A virus-infected F508del CFTR-heterozygous mice.
Cystic Fibrosis
TGF-?1 Augments the Apical Membrane Abundance of Lemur Tyrosine Kinase 2 to Inhibit CFTR-Mediated Chloride Transport in Human Bronchial Epithelia.
Cystic Fibrosis
TGF-beta 1 and Cigarette Smoke Inhibit the Ability of ?2-agonists to Enhance Epithelial Permeability.
Cystic Fibrosis
Tgf-beta downregulation of distinct chloride channels in cystic fibrosis-affected epithelia.
Cystic Fibrosis
TGF-beta(1) genotype and accelerated decline in lung function of patients with cystic fibrosis.
Cystic Fibrosis
TGFbeta down-regulation of the CFTR: a means to limit epithelial chloride secretion.
Cystic Fibrosis
The 2588G-->C mutation in the ABCR gene is a mild frequent founder mutation in the Western European population and allows the classification of ABCR mutations in patients with Stargardt disease.
Cystic Fibrosis
The 3120 +1G-->A splicing mutation in CFTR is common in Brazilian cystic fibrosis patients.
Cystic Fibrosis
The 8.1 ancestral MHC haplotype is associated with delayed onset of colonization in cystic fibrosis.
Cystic Fibrosis
The ?F508 Gene Mutation of Cystic Fibrosis Transmembrane Regulator Protein Leads to a Progressive Decline of Beta-Cell Function in Mice Carrying This Mutation.
Cystic Fibrosis
The ?F508 Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator Is Associated With Progressive Insulin Resistance and Decreased Functional ?-Cell Mass in Mice.
Cystic Fibrosis
The actin filament disrupter cytochalasin D activates the recombinant cystic fibrosis transmembrane conductance regulator Cl- channel in mouse 3T3 fibroblasts.
Cystic Fibrosis
The acute and regulatory phases of time-course changes in gill mitochondrion-rich cells of seawater-acclimated medaka (Oryzias dancena) when exposed to hypoosmotic environments.
Cystic Fibrosis
The adenosine 2A receptor agonist GW328267C improves lung function after acute lung injury in rats.
Cystic Fibrosis
The adenosine A2B receptor is involved in anion secretion in human pancreatic duct Capan-1 epithelial cells.
Cystic Fibrosis
The alpha2beta1 isoform of guanylyl cyclase mediates plasma membrane localized nitric oxide signalling.
Cystic Fibrosis
The amiloride-inhibitable Na+ conductance is reduced by the cystic fibrosis transmembrane conductance regulator in normal but not in cystic fibrosis airways.
Cystic Fibrosis
The amino-terminal cyclic nucleotide binding site of the type II cGMP-dependent protein kinase is essential for full cyclic nucleotide-dependent activation.
Cystic Fibrosis
The anion transporter SLC26A9 localizes to tight junctions and is degraded by the proteasome when co-expressed with F508del-CFTR.
Cystic Fibrosis
The antigen-binding characteristics of mAbs derived from in vivo priming of avian B cells.
Cystic Fibrosis
The apical anion exchanger Slc26a6 promotes oxalate secretion by murine submandibular gland acinar cells.
Cystic Fibrosis
The Application of Bicarbonate Recovers the Chemical-Physical Properties of Airway Surface Liquid in Cystic Fibrosis Epithelia Models.
Cystic Fibrosis
The application of telemedicine in the follow-up of lung transplantation in a patient with cystic fibrosis.
Cystic Fibrosis
The ATP-binding component of a prokaryotic traffic ATPase is exposed to the periplasmic (external) surface.
Cystic Fibrosis
The Balance between the Safety of Mother, Fetus, and Newborn Undergoing Cystic Fibrosis Transmembrane Conductance Regulator Treatments during Pregnancy.
Cystic Fibrosis
The bioflavonoid compound, sinupret, stimulates transepithelial chloride transport in vitro and in vivo.
Cystic Fibrosis
The biogenesis, traffic, and function of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
The C-terminal part of the R-domain, but not the PDZ binding motif, of CFTR is involved in interaction with Ca(2+)-activated Cl- channels.
Cystic Fibrosis
The C-terminus of nucleotide binding domain 1 contains critical features for cystic fibrosis transmembrane conductance regulator trafficking and activation.
Cystic Fibrosis
The calpain, caspase 12, caspase 3 cascade leading to apoptosis is altered in F508del-CFTR expressing cells.
Cystic Fibrosis
The carboxyl terminus of the cystic fibrosis transmembrane conductance regulator binds to AP-2 clathrin adaptors.
Cystic Fibrosis
The cellular localization of Na(+)/H(+) exchanger 1, cystic fibrosis transmembrane conductance regulator, potassium channel, epithelial sodium channel ? and vacuolar-type H+-ATPase in human eccrine sweat glands.
Cystic Fibrosis
The CF-modifying gene EHF promotes p.Phe508del-CFTR residual function by altering protein glycosylation and trafficking in epithelial cells.
Cystic Fibrosis
The CFTR M470V gene variant as a potential modifier of COPD severity: study of Serbian population.
Cystic Fibrosis
The CFTR M470V, intron 8 poly-T, and 8 TG-repeats detection in Chinese males with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
The CFTR Met 470 allele is associated with lower birth rates in fertile men from a population isolate.
Cystic Fibrosis
The CFTR Mutation c.3453G > C (D1152H) Confers an Anion Selectivity Defect in Primary Airway Tissue that Can Be Rescued by Ivacaftor.
Cystic Fibrosis
The CFTR P67L variant reveals a key role for N-terminal lasso helices in channel folding, maturation, and pharmacologic rescue.
Cystic Fibrosis
The CFTR polymorphisms poly-T, TG-repeats and M470V in Chinese males with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
The CFTR-Associated Ligand Arrests the Trafficking of the Mutant ?F508 CFTR Channel in the ER Contributing to Cystic Fibrosis.
Cystic Fibrosis
The CFTR-derived peptides as a model of sequence-specific protein aggregation.
Cystic Fibrosis
The changing face of the exocrine pancreas in cystic fibrosis: pancreatic sufficiency, pancreatitis and genotype.
Cystic Fibrosis
The chloride channel CFTR controls cellular quiescence by hyperpolarizing the cell membrane during diapause in the crustacean Artemia.
Cystic Fibrosis
The chloride channel CFTR is not required for cyst growth in an ADPKD mouse model.
Cystic Fibrosis
The chloride channel ClC-4 co-localizes with cystic fibrosis transmembrane conductance regulator and may mediate chloride flux across the apical membrane of intestinal epithelia.
Cystic Fibrosis
The classification of ATP-binding cassette subfamily A member 3 mutations using the cystic fibrosis transmembrane conductance regulator classification system.
Cystic Fibrosis
The CLIC1 chloride channel is regulated by the cystic fibrosis transmembrane conductance regulator when expressed in Xenopus oocytes.
Cystic Fibrosis
The Clinical Biology of Cystic Fibrosis Transmembrane Regulator Protein: Its Role and Function in Extrapulmonary Disease.
Cystic Fibrosis
The cochaperone HspBP1 inhibits the CHIP ubiquitin ligase and stimulates the maturation of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment.
Cystic Fibrosis
The complex relationships between cystic fibrosis and congenital bilateral absence of the vas deferens: clinical, electrophysiological and genetic data.
Cystic Fibrosis
The course of glucose intolerance in children with cystic fibrosis: a retrospective study - preliminary report.
Cystic Fibrosis
The cubicon method for concentrating membrane proteins in the cubic mesophase.
Cystic Fibrosis
The CYSMA web server: An example of integrative tool for in silico analysis of missense variants identified in Mendelian disorders.
Cystic Fibrosis
The cystic fibrosis conductance regulator gene exon sequence is normal in a patient with edematous eosinophilic nasal polyps.
Cystic Fibrosis
The cystic fibrosis mutation (delta F508) does not influence the chloride channel activity of CFTR.
Cystic Fibrosis
The cystic fibrosis mutation G1349D within the signature motif LSHGH of NBD2 abolishes the activation of CFTR chloride channels by genistein.
Cystic Fibrosis
The cystic fibrosis mutation G551D alters the non-Michaelis-Menten behavior of the cystic fibrosis transmembrane conductance regulator (CFTR) channel and abolishes the inhibitory Genistein binding site.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator (CFTR) and chloride-dependent ion fluxes of ovine vocal fold epithelium.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator (CFTR) and its stability.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator (CFTR) inhibits ENaC through an increase in the intracellular Cl- concentration.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in maturation stage ameloblasts, odontoblasts and bone cells.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator (Cftr) modulates the timing of puberty in mice.
Cystic Fibrosis
The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Uses its C-Terminus to Regulate the A2B Adenosine Receptor.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator (CFTR): three-dimensional structure and localization of a channel gate.
Cystic Fibrosis
The Cystic Fibrosis Transmembrane Conductance Regulator 470 Met Allele Is Associated with an Increased Risk of Chronic Pancreatitis in Both Asian and Caucasian Populations: A Meta-Analysis.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator activates aquaporin 3 in airway epithelial cells.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator and its function in epithelial transport.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator as a biomarker in non-small cell lung cancer.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator as a marker of human pancreatic duct development.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator attenuates the endogenous Ca2+ activated Cl- conductance of Xenopus oocytes.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator chloride channel. Iodide block and permeation.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator Cl(-) channel: a versatile engine for transepithelial ion transport.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator gene and ion channel function in patients with idiopathic pancreatitis.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator impedes proteolytic stimulation of the epithelial Na+ channel.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator in reproductive health and disease.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator interacts with and regulates the activity of the HCO3- salvage transporter human Na+-HCO3- cotransport isoform 3.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator interacts with multiple Ig domains of filamin A.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator is a dual ATP and chloride channel.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator is not a base transporter in isolated duodenal epithelial cells.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator is regulated by a direct interaction with the protein phosphatase 2A.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator mediates transepithelial fluid secretion by human autosomal dominant polycystic kidney disease epithelium in vitro.
Cystic Fibrosis
The Cystic Fibrosis Transmembrane Conductance Regulator's Expanding SNARE Interactome.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator. Nucleotide binding to a synthetic peptide segment from the second predicted nucleotide binding fold.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator. Overexpression, purification, and characterization of wild type and delta F508 mutant forms of the first nucleotide binding fold in fusion with the maltose-binding protein.
Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator: an intriguing protein with pleiotropic functions.
Cystic Fibrosis
The cystic fibrosis transmembrane recruiter the alter ego of CFTR as a multi-kinase anchor.
Cystic Fibrosis
The cystic fibrosis transmembrane regulator forms macromolecular complexes with PDZ domain scaffold proteins.
Cystic Fibrosis
The cystic fibrosis transmembrane regulator gene is expressed in the human endocervix throughout the menstrual cycle.
Cystic Fibrosis
The cystic fibrosis transmembrane regulator is present and functional in endosomes. Role as a determinant of endosomal pH.
Cystic Fibrosis
The cystic fibrosis-causing mutation {Delta}F508 affects multiple steps in CFTR biogenesis.
Cystic Fibrosis
The cystic-fibrosis-associated {Delta}F508 mutation confers post-transcriptional destabilization on the C. elegans ABC transporter PGP-3.
Cystic Fibrosis
The cytosolic termini of the beta- and gamma-ENaC subunits are involved in the functional interactions between cystic fibrosis transmembrane conductance regulator and epithelial sodium channel.
Cystic Fibrosis
The decline of fertility in male uremic patients is correlated with low expression of the cystic fibrosis transmembrane conductance regulator protein (CFTR) in human sperm.
Cystic Fibrosis
The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells.
Cystic Fibrosis
The Delta F508 mutation shortens the biochemical half-life of plasma membrane CFTR in polarized epithelial cells.
Cystic Fibrosis
The DeltaF508 mutation disrupts packing of the transmembrane segments of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
The deltaF508 mutation in the cystic fibrosis transmembrane conductance regulator alters control of essential fatty acid utilization in epithelial cells.
Cystic Fibrosis
The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colon.
Cystic Fibrosis
The detection and significance of cystic fibrosis transmembrane conductance regulator gene promoter mutations in Chinese patients with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
The deubiquitinating enzyme USP10 regulates the endocytic recycling of CFTR in airway epithelial cells.
Cystic Fibrosis
The deubiquitinating enzyme USP10 regulates the post-endocytic sorting of CFTR in airway epithelial cells.
Cystic Fibrosis
The differential role of renoguanylin in osmoregulation and apical Cl-/HCO3- exchange activity in the posterior intestine of the Gulf toadfish (Opsanus beta).
Cystic Fibrosis
The direct early diagnosis of cystic fibrosis by the detection of the delta F508 CFTR gene mutation in a prematurely delivered boy.
Cystic Fibrosis
The distribution and expression of CFTR restricts electrogenic anion secretion to the ileum of the brushtail possum, Trichosurus vulpecula.
Cystic Fibrosis
The effect of ambroxol on chloride transport, CFTR and ENaC in cystic fibrosis airway epithelial cells.
Cystic Fibrosis
The effect of chloride concentration on human neutrophil functions: potential relevance to cystic fibrosis.
Cystic Fibrosis
The effect of environmental salinity on the protein expression of Na+/K+-ATPase, Na+/K+/2Cl- cotransporter, cystic fibrosis transmembrane conductance regulator, anion exchanger 1, and chloride channel 3 in gills of a euryhaline teleost, Tetraodon nigroviridis.
Cystic Fibrosis
The Effect of Sodium Bicarbonate, a Beneficial Adjuvant Molecule in Cystic Fibrosis, on Bronchial Epithelial Cells Expressing a Wild-Type or Mutant CFTR Channel.
Cystic Fibrosis
The Effect of Synonymous Single-Nucleotide Polymorphisms on an Atypical Cystic Fibrosis Clinical Presentation.
Cystic Fibrosis
The effect of varying tonicity on nasal epithelial ion transport in cystic fibrosis.
Cystic Fibrosis
The effects induced by the sulphonylurea glibenclamide on the neonatal rat spinal cord indicate a novel mechanism to control neuronal excitability and inhibitory neurotransmission.
Cystic Fibrosis
The effects of acute salinity challenges on osmoregulation in Mozambique tilapia reared in a tidally changing salinity.
Cystic Fibrosis
The effects of transfer from steady-state to tidally-changing salinities on plasma and branchial osmoregulatory variables in adult Mozambique tilapia.
Cystic Fibrosis
The endoplasmic reticulum-associated Hsp40 DNAJB12 and Hsc70 cooperate to facilitate RMA1 E3-dependent degradation of nascent CFTRDeltaF508.
Cystic Fibrosis
The Enigmatic Gut in Cystic Fibrosis: Linking Inflammation, Dysbiosis, and the Increased Risk of Malignancy.
Cystic Fibrosis
The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis lung disease.
Cystic Fibrosis
The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis.
Cystic Fibrosis
The ER-resident ubiquitin-specific protease 19 participates in the UPR and rescues ERAD substrates.
Cystic Fibrosis
The Etiology and Clinical Course of Chronic Pancreatitis in Children With Early Onset of the Disease.
Cystic Fibrosis
The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis.
Cystic Fibrosis
The expression of three topologically distinct membrane proteins elicits unique stress response pathways in the yeast Saccharomyces cerevisiae.
Cystic Fibrosis
The extracellular calcium-sensing receptor regulates human fetal lung development via CFTR.
Cystic Fibrosis
The Extrapulmonary Effects of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Cystic Fibrosis.
Cystic Fibrosis
The F508del mutation in cystic fibrosis transmembrane conductance regulator gene impacts bone formation.
Cystic Fibrosis
The Fifth Transmembrane Segment of Cystic Fibrosis Transmembrane Conductance Regulator Contributes to Its Anion Permeation Pathway.
Cystic Fibrosis
The First Nucleotide Binding Domain of Cystic Fibrosis Transmembrane Conductance Regulator Is a Site of Stable Nucleotide Interaction, whereas the Second Is a Site of Rapid Turnover.
Cystic Fibrosis
The first nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator can function as an active ATPase.
Cystic Fibrosis
The First Successful Lung Transplantation in a Korean Child with Cystic Fibrosis.
Cystic Fibrosis
The formation of the cAMP/protein kinase A-dependent annexin 2 S100A10 complex with cystic fibrosis conductance regulator protein (CFTR) regulates CFTR channel function.
Cystic Fibrosis
The function of the intermediate compartment in pre-Golgi trafficking involves its stable connection with the centrosome.
Cystic Fibrosis
The gasotransmitter hydrogen sulfide inhibits transepithelial anion secretion of pregnant mouse endometrial epithelium.
Cystic Fibrosis
The gene for congenital chloride diarrhea maps close to but is distinct from the gene for cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
The genetic background of osteoporosis in cystic fibrosis: association analysis with polymorphic markers in four candidate genes.
Cystic Fibrosis
The gliadin-CFTR connection: new perspectives for the treatment of celiac disease.
Cystic Fibrosis
The glycine residues G551 and G1349 within the ATP-binding cassette signature motifs play critical roles in the activation and inhibition of cystic fibrosis transmembrane conductance regulator channels by phloxine B.
Cystic Fibrosis
The H-loop in the second nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator is required for efficient chloride channel closing.
Cystic Fibrosis
The HDAC inhibitor SAHA does not rescue CFTR membrane expression in Cystic Fibrosis.
Cystic Fibrosis
The hsa-miR-125a/hsa-let-7e/hsa-miR-99b cluster is potentially implicated in Cystic Fibrosis pathogenesis.
Cystic Fibrosis
The human CFTR protein expressed in CHO cells activates aquaporin-3 in a cAMP-dependent pathway: study by digital holographic microscopy.
Cystic Fibrosis
The human DnaJ homologue (Hdj)-1/heat-shock protein (Hsp) 40 co-chaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70.
Cystic Fibrosis
The hypertonic environment differentially regulates wild-type CFTR and TNR-CFTR chloride channels.
Cystic Fibrosis
The ichthyotoxic alga Chattonella marina induces Na+, K+ -ATPase, and CFTR proteins expression in fish gill chloride cells in vivo.
Cystic Fibrosis
The impact of Cystic Fibrosis Transmembrane Regulator Disruption on cardiac function and stress response.
Cystic Fibrosis
The impact of ivacaftor on sinonasal pathology in S1251N-mediated cystic fibrosis patients.
Cystic Fibrosis
The Impact of Secondhand Smoke Exposure on Children with Cystic Fibrosis: A Review.
Cystic Fibrosis
The Impact of the CFTR Gene Discovery on Cystic Fibrosis Diagnosis, Counseling, and Preventive Therapy.
Cystic Fibrosis
The Impact on Genetic Testing of Mutational Patterns of CFTR Gene in Different Clinical Macrocategories of Cystic Fibrosis.
Cystic Fibrosis
The impacts of along-channel acupuncture on the protein expressions of the chloride channel of the rats with myocardial ischemia.
Cystic Fibrosis
The implications and management of cystic fibrosis screen positive, inconclusive diagnosis patients.
Cystic Fibrosis
The implications of CFTR structural studies for cystic fibrosis drug development.
Cystic Fibrosis
The inhibition mechanism of non-phosphorylated Ser768 in the regulatory domain of cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
The inhibitory role of purinergic P2Y receptor on Mg2+ transport across intestinal epithelium-like Caco-2 monolayer.
Cystic Fibrosis
The inner opercular membrane of the euryhaline teleost: a useful surrogate model for comparisons of different characteristics of ionocytes between seawater- and freshwater-acclimated medaka.
Cystic Fibrosis
The interaction between syntaxin 1A and cystic fibrosis transmembrane conductance regulator Cl- channels is mechanistically distinct from syntaxin 1A-SNARE interactions.
Cystic Fibrosis
The Intestinal Microbiome and Cystic Fibrosis Transmembrane Conductance Regulator Modulators: Emerging Themes in the Management of Gastrointestinal Manifestations of Cystic Fibrosis.
Cystic Fibrosis
The investigational Cystic Fibrosis drug Trimethylangelicin directly modulates CFTR by stabilizing the first membrane-spanning domain.
Cystic Fibrosis
The IP3 R Binding Protein Released With Inositol 1,4,5-Trisphosphate Is Expressed in Rodent Reproductive Tissue and Spermatozoa.
Cystic Fibrosis
The KDEL receptor has a role in the biogenesis and trafficking of the epithelial sodium channel (ENaC).
Cystic Fibrosis
The L441P mutation of cystic fibrosis transmembrane conductance regulator and its molecular pathogenic mechanisms in a Korean patient with cystic fibrosis.
Cystic Fibrosis
The lactoperoxidase system links anion transport to host defense in cystic fibrosis.
Cystic Fibrosis
The lamellae-free-type pseudobranch of the euryhaline milkfish (Chanos chanos) is a Na(+), K(+)-ATPase-abundant organ involved in hypoosmoregulation.
Cystic Fibrosis
The low PLC-?1 expression in cystic fibrosis bronchial epithelial cells induces upregulation of TRPV6 channel activity.
Cystic Fibrosis
The mechanism of cystic fibrosis transmembrane conductance regulator transcriptional repression during the unfolded protein response.
Cystic Fibrosis
The mechanism underlying cystic fibrosis transmembrane conductance regulator transport from the endoplasmic reticulum to the proteasome includes Sec61beta and a cytosolic, deglycosylated intermediary.
Cystic Fibrosis
The Mechanistic Links between Insulin and Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Cl(-) Channel.
Cystic Fibrosis
The membrane-bound bile acid receptor TGR5 is localized in the epithelium of human gallbladders.
Cystic Fibrosis
The MglA component of the binding protein-dependent galactose transport system of Salmonella typhimurium is a galactose-stimulated ATPase.
Cystic Fibrosis
The mitochondrial Complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function.
Cystic Fibrosis
The molecular basis of partial penetrance of splicing mutations in cystic fibrosis.
Cystic Fibrosis
The molecular chaperone Hsc70 assists the in vitro folding of the N-terminal nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
The molecular mechanism of CFTR- and secretin-dependent renal bicarbonate excretion.
Cystic Fibrosis
The most common cystic fibrosis-associated mutation destabilizes the dimeric state of the nucleotide-binding domains of CFTR.
Cystic Fibrosis
The multi-herbal drug STW 5 (Iberogast(R)) has prosecretory action in the human intestine.
Cystic Fibrosis
The mycotoxin ochratoxin A alters intestinal barrier and absorption functions but has no effect on chloride secretion.
Cystic Fibrosis
The Na(+)/H(+) exchanger regulatory factor 2 mediates phosphorylation of serum- and glucocorticoid-induced protein kinase 1 by 3-phosphoinositide-dependent protein kinase 1.
Cystic Fibrosis
The necessity of complete CFTR mutational analysis of an infertile couple before in vitro fertilization.
Cystic Fibrosis
The need for vigilance: the case of a false-negative newborn screen for cystic fibrosis.
Cystic Fibrosis
The neglected biliary mucus and its phosphatidylcholine content: a putative player in pathogenesis of primary cholangitis-a narrative review article.
Cystic Fibrosis
The non-hydrolytic pathway of cystic fibrosis transmembrane conductance regulator ion channel gating.
Cystic Fibrosis
The Novel CFTR Mutation A457P in a Male with a Delayed Diagnosis of Cystic Fibrosis.
Cystic Fibrosis
The novel complex allele [A238V;F508del] of the CFTR gene: clinical phenotype and possible implications for cystic fibrosis etiological therapies.
Cystic Fibrosis
The nucleotide binding folds of the cystic fibrosis transmembrane conductance regulator are extracellularly accessible.
Cystic Fibrosis
The occurrence of various non-delta F508 CFTR gene mutations among Hungarian cystic fibrosis patients.
Cystic Fibrosis
The odorant receptor OR2W3 on airway smooth muscle evokes bronchodilation via a cooperative chemosensory tradeoff between TMEM16A and CFTR.
Cystic Fibrosis
The optimal approach to nutrition and cystic fibrosis: latest evidence and recommendations.
Cystic Fibrosis
The origin of and subcellular mechanisms causing pancreatic bicarbonate secretion.
Cystic Fibrosis
The osmoregulatory effects of rearing Mozambique tilapia in a tidally changing salinity.
Cystic Fibrosis
The pancreatitis-associated protein (PAP). A new candidate for neonatal screening of cystic fibrosis.
Cystic Fibrosis
The patch-clamp and planar lipid bilayer techniques: powerful and versatile tools to investigate the CFTR Cl- channel.
Cystic Fibrosis
The PDZ-interacting domain of cystic fibrosis transmembrane conductance regulator is required for functional expression in the apical plasma membrane.
Cystic Fibrosis
The PEST sequence does not contribute to the stability of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
The phorbol ester PMA and cyclic AMP activate different Cl(-) and HCO3(-) fluxes in C127 cells expressing CFTR.
Cystic Fibrosis
The Phosphodiesterase Inhibitor Ensifentrine Reduces Production of Proinflammatory Mediators in Well Differentiated Bronchial Epithelial Cells by Inhibiting PDE4.
Cystic Fibrosis
The phospholipid flippase ATP8B1 mediates apical localization of the cystic fibrosis transmembrane regulator.
Cystic Fibrosis
The Physiology and Pathophysiology of Pancreatic Ductal Secretion: The Background for Clinicians.
Cystic Fibrosis
The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation.
Cystic Fibrosis
The pore architecture of the cystic fibrosis transmembrane conductance regulator channel revealed by co-mutation in pore-forming transmembrane regions.
Cystic Fibrosis
The possible additional role of the cystic fibrosis transmembrane regulator to motoneuron inhibition produced by glycine effects.
Cystic Fibrosis
The Potential of Wharton's Jelly Derived Mesenchymal Stem Cells in Treating Patients with Cystic Fibrosis.
Cystic Fibrosis
The Potential Role and Regulatory Mechanisms of MUC5AC in Chronic Obstructive Pulmonary Disease.
Cystic Fibrosis
The power stroke driven by ATP binding in CFTR as studied by molecular dynamics simulations.
Cystic Fibrosis
The preclinical discovery and development of the combination of ivacaftor + tezacaftor used to treat cystic fibrosis.
Cystic Fibrosis
The predictive potential of the sweat chloride test in cystic fibrosis patients with the G551D mutation.
Cystic Fibrosis
The promoter of the human cystic fibrosis transmembrane conductance regulator gene directing SV40 T antigen expression induces malignant proliferation of ependymal cells in transgenic mice.
Cystic Fibrosis
The protein kinase A-regulated cardiac Cl- channel resembles the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
The protein tyrosine kinase pathway is not involved in the regulation of K+ transport across the rat colon.
Cystic Fibrosis
The proteome speciation of an immortalized cystic fibrosis cell line: New perspectives on the pathophysiology of the disease.
Cystic Fibrosis
The Pseudomonas aeruginosa secreted protein PA2934 decreases apical membrane expression of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
The rad50 signature motif: essential to ATP binding and biological function.
Cystic Fibrosis
The regulatory role of vasoactive intestinal peptide in lacrimal gland ductal fluid secretion: A new piece of the puzzle in tear production.
Cystic Fibrosis
The relationship between cell proliferation, Cl- secretion, and renal cyst growth: a study using CFTR inhibitors.
Cystic Fibrosis
The relationship between genotype and exercise tolerance in children with cystic fibrosis.
Cystic Fibrosis
The relationship of chronic mucin secretion to airway disease in normal and CFTR-deficient mice.
Cystic Fibrosis
The relative binding affinities of PDZ partners for CFTR: a biochemical basis for efficient endocytic recycling.
Cystic Fibrosis
The relevance of sweat testing for the diagnosis of cystic fibrosis in the genomic era.
Cystic Fibrosis
The role of chloride anion and CFTR in killing of Pseudomonas aeruginosa by normal and CF neutrophils.
Cystic Fibrosis
The role of common single-nucleotide polymorphisms on exon 9 and exon 12 skipping in nonmutated CFTR alleles.
Cystic Fibrosis
The role of cystic fibrosis transmembrane conductance regulator chloride channel in beta-receptor-mediated regulation of alveolar fluid clearance.
Cystic Fibrosis
The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating.
Cystic Fibrosis
The role of epithelial P2Y2 and P2Y4 receptors in the regulation of intestinal chloride secretion.
Cystic Fibrosis
The role of local angiotensins and prostaglandins in the control of anion secretion by the rat epididymis.
Cystic Fibrosis
The role of nuclear factor E2-Related factor 2 and uncoupling protein 2 in glutathione metabolism: Evidence from an in vivo gene knockout study.
Cystic Fibrosis
The role of SLC9A3 in Taiwanese patients with congenital bilateral absence of vas deferens (CBAVD).
Cystic Fibrosis
The Role of Specialized Pro-Resolving Mediators in Cystic Fibrosis Airways Disease.
Cystic Fibrosis
The role of sphingolipids and ceramide in pulmonary inflammation in cystic fibrosis.
Cystic Fibrosis
The role of the C terminus and Na+/H+ exchanger regulatory factor in the functional expression of cystic fibrosis transmembrane conductance regulator in nonpolarized cells and epithelia.
Cystic Fibrosis
The role of the F508C mutation in congenital bilateral absence of the vas deferens.
Cystic Fibrosis
The ROMK-cystic fibrosis transmembrane conductance regulator connection: new insights into the relationship between ROMK and cystic fibrosis transmembrane conductance regulator channels.
Cystic Fibrosis
The safety and efficacy of oral docosahexaenoic acid supplementation for the treatment of primary sclerosing cholangitis - a pilot study.
Cystic Fibrosis
The safety of lumacaftor and ivacaftor for the treatment of cystic fibrosis.
Cystic Fibrosis
The SbcCD protein of Escherichia coli is related to two putative nucleases in the UvrA superfamily of nucleotide-binding proteins.
Cystic Fibrosis
The second half of the cystic fibrosis transmembrane conductance regulator forms a functional chloride channel.
Cystic Fibrosis
The self-interaction of native TDP-43 C terminus inhibits its degradation and contributes to early proteinopathies.
Cystic Fibrosis
THE SHARK RECTAL GLAND MODEL: A CHAMPION OF RECEPTOR MEDIATED CHLORIDE SECRETION THROUGH CFTR.
Cystic Fibrosis
The sheep genome contributes to localization of control elements in a human gene with complex regulatory mechanisms.
Cystic Fibrosis
The short apical membrane half-life of rescued {Delta}F508-cystic fibrosis transmembrane conductance regulator (CFTR) results from accelerated endocytosis of {Delta}F508-CFTR in polarized human airway epithelial cells.
Cystic Fibrosis
The short-term effects of ORKAMBI (lumacaftor/ivacaftor) on regional and distal lung structures using functional respiratory imaging.
Cystic Fibrosis
The silent codon change I507-ATC->ATT contributes to the severity of the ?F508 CFTR channel dysfunction.
Cystic Fibrosis
The Slc26a4 transporter functions as an electroneutral Cl-/I-/HCO3- exchanger: role of Slc26a4 and Slc26a6 in I- and HCO3- secretion and in regulation of CFTR in the parotid duct.
Cystic Fibrosis
The Small GTPase Rab11b Regulates Degradation of Surface Membrane L-Type Cav1.2 Channels.
Cystic Fibrosis
The spectrum of CFTR mutations in south-west German cystic fibrosis patients.
Cystic Fibrosis
The study of cystic fibrosis transmembrane conductance regulator gene mutations in a group of patients from Romania.
Cystic Fibrosis
The substituted benzimidazolone NS004 is an opener of the cystic fibrosis chloride channel.
Cystic Fibrosis
The suitability of matrix assisted laser desorption/ionization time of flight mass spectrometry in a laboratory developed test using cystic fibrosis carrier screening as a model.
Cystic Fibrosis
The susceptibility of T5-TG12 of the CFTR gene in chronic bronchitis occurrence in a Chinese population in Jiangsu province, China.
Cystic Fibrosis
The Sweat Metabolome of Screen-Positive Cystic Fibrosis Infants: Revealing Mechanisms beyond Impaired Chloride Transport.
Cystic Fibrosis
The swelling-activated anion conductance in the mouse renal inner medullary collecting duct cell line mIMCD-K2.
Cystic Fibrosis
The TAK1?IKK??TPL2?MKK1/MKK2 Signaling Cascade Regulates IL-33 Expression in Cystic Fibrosis Airway Epithelial Cells Following Infection by Pseudomonas aeruginosa.
Cystic Fibrosis
The testis anion transporter TAT1 (SLC26A8) physically and functionally interacts with the cystic fibrosis transmembrane conductance regulator channel: a potential role during sperm capacitation.
Cystic Fibrosis
The therapeutic potential of the human cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
The transmembrane protein CBP plays a role in transiently anchoring small clusters of Thy-1, a GPI-anchored protein, to the cytoskeleton.
Cystic Fibrosis
The Two ATP Binding Sites of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Play Distinct Roles in Gating Kinetics and Energetics.
Cystic Fibrosis
The two nucleotide-binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) have distinct functions in controlling channel activity.
Cystic Fibrosis
The type IVB pili of Salmonella enterica serovar Typhi bind to the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
The tyrosine kinase p60c-src regulates the fast gate of the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
The U.S. Food and Drug Administration's Experience with Ivacaftor in Cystic Fibrosis. Establishing Efficacy Using In Vitro Data in Lieu of a Clinical Trial.
Cystic Fibrosis
The Ubiquitin Ligase E6-AP Is Induced and Recruited to Aggresomes in Response to Proteasome Inhibition and May Be Involved in the Ubiquitination of Hsp70-bound Misfolded Proteins.
Cystic Fibrosis
The uroguanylin gene (Guca1b) is linked to guanylin (Guca2) on mouse chromosome 4.
Cystic Fibrosis
The use of DHPLC (Denaturing High Performance Liquid Chromatography) in II level screening of the CFTR gene in Prenatal Diagnosis.
Cystic Fibrosis
The use of ivacaftor in CFTR mutations resulting in residual functioning protein.
Cystic Fibrosis
The use of lumacaftor/ivacaftor to treat acute deterioration in paediatric cystic fibrosis.
Cystic Fibrosis
The winged helix transcription factor HFH-4 is expressed during choroid plexus epithelial development in the mouse embryo.
Cystic Fibrosis
The yin and yang of cystic fibrosis transmembrane conductance regulator function: implications for chronic lung disease.
Cystic Fibrosis
The zebrafish Kupffer's vesicle as a model system for the molecular mechanisms by which the lack of Polycystin-2 leads to stimulation of CFTR.
Cystic Fibrosis
The {Delta}F508 Mutation Causes CFTR Misprocessing and Cystic Fibrosis-Like Disease in Pigs.
Cystic Fibrosis
Therapeutic Approaches to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis.
Cystic Fibrosis
Therapeutic approaches to CFTR dysfunction: From discovery to drug development.
Cystic Fibrosis
Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting.
Cystic Fibrosis
Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation.
Cystic Fibrosis
Therapeutic challenges posed by critical drug-drug interactions in cystic fibrosis.
Cystic Fibrosis
Therapeutic peptides for the treatment of cystic fibrosis: Challenges and perspectives.
Cystic Fibrosis
Therapeutic pipeline for individuals with cystic fibrosis with mutations nonresponsive to current cystic fibrosis transmembrane conductance regulator modulators.
Cystic Fibrosis
Therapeutic Potential for CFTR Correctors in Autosomal Recessive Polycystic Kidney Disease.
Cystic Fibrosis
Therapeutic potential of cystic fibrosis transmembrane conductance regulator (CFTR) inhibitors in polycystic kidney disease.
Cystic Fibrosis
Therapeutic potential of serum and glucocorticoid inducible kinase inhibition.
Cystic Fibrosis
Therapeutically Targeting Tumor Necrosis Factor-?/Sphingosine-1-Phosphate Signaling Corrects Myogenic Reactivity in Subarachnoid Hemorrhage.
Cystic Fibrosis
Theratyping cystic fibrosis in vitro in ALI-culture and organoid models generated from patient-derived nasal epithelial Conditionally Reprogrammed Stem Cells.
Cystic Fibrosis
Thermal instability of ?F508 cystic fibrosis transmembrane conductance regulator (CFTR) channel function: protection by single suppressor mutations and inhibiting channel activity.
Cystic Fibrosis
Thermodynamic study of the native and phosphorylated regulatory domain of the CFTR.
Cystic Fibrosis
Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion.
Cystic Fibrosis
Thiazolidinone CFTR inhibitors with improved water solubility identified by structure-activity analysis.
Cystic Fibrosis
Thiocyanate as a probe of the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Cystic Fibrosis
Thiocyanate transport in resting and IL-4-stimulated human bronchial epithelial cells: role of pendrin and anion channels.
Cystic Fibrosis
Thixotropic solutions enhance viral-mediated gene transfer to airway epithelia.
Cystic Fibrosis
Three novel mutations (I506S, S466X, 1651A-->T) in exon 10 of the cystic fibrosis transmembrane conductance regulator (CFTR) detected in patients of southern German descent.
Cystic Fibrosis
Three novel sequence variations in the 5' upstream region of the cystic fibrosis transmembrane conductance regulator (CFTR) gene: two polymorphisms and one putative molecular defect.
Cystic Fibrosis
Three point mutations in the CFTR gene in French cystic fibrosis patients: identification by denaturing gradient gel electrophoresis.
Cystic Fibrosis
Three-dimensional reconstruction of human cystic fibrosis transmembrane conductance regulator chloride channel revealed an ellipsoidal structure with orifices beneath the putative transmembrane domain.
Cystic Fibrosis
Thymosin ?-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia.
Cystic Fibrosis
Thymosin ?1 represents a potential potent single-molecule-based therapy for cystic fibrosis.
Cystic Fibrosis
Thymosin alpha 1 exerts beneficial extrapulmonary effects in cystic fibrosis.
Cystic Fibrosis
Time course of salinity adaptation in a strongly euryhaline estuarine teleost, fundulus heteroclitus: a multivariable approach
Cystic Fibrosis
Time for a gut check: Pancreatic sufficiency resulting from CFTR modulator use.
Cystic Fibrosis
Timing and sequence of differentiation of embryonic rat hepatocytes along the biliary epithelial lineage.
Cystic Fibrosis
Tissue-specific in vivo transcription start sites of the human and murine cystic fibrosis genes.
Cystic Fibrosis
Tissue-specific modulation of glucocorticoid receptor expression in response to salinity acclimation in rainbow trout.
Cystic Fibrosis
TMEM16A: An Alternative Approach to Restoring Airway Anion Secretion in Cystic Fibrosis?
Cystic Fibrosis
Tobacco carcinogen NNK transporter MRP2 regulates CFTR function in lung epithelia: implications for lung cancer.
Cystic Fibrosis
Tolbutamide causes open channel blockade of cystic fibrosis transmembrane conductance regulator Cl- channels.
Cystic Fibrosis
Toll-like receptor-4 genotype influences the survival of cystic fibrosis mice.
Cystic Fibrosis
Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.
Cystic Fibrosis
Topogenesis of cystic fibrosis transmembrane conductance regulator (CFTR): regulation by the amino terminal transmembrane sequences.
Cystic Fibrosis
Topological model of membrane domain of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Toward an animal model of cystic fibrosis: targeted interruption of exon 10 of the cystic fibrosis transmembrane regulator gene in embryonic stem cells.
Cystic Fibrosis
Towards next generation therapies for cystic fibrosis: Folding, function and pharmacology of CFTR.
Cystic Fibrosis
Tracking of quantum dot-labeled CFTR shows near immobilization by C-terminal PDZ interactions.
Cystic Fibrosis
Traditional Chinese formula, lubricating gut pill, improves loperamide-induced rat constipation involved in enhance of Cl- secretion across distal colonic epithelium.
Cystic Fibrosis
Traffic pattern of cystic fibrosis transmembrane regulator through the early exocytic pathway.
Cystic Fibrosis
Traffic-independent function of the Sar1p/COPII machinery in proteasomal sorting of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Trafficking and Function of the Cystic Fibrosis Transmembrane Conductance Regulator: A Complex Network of Post-Translational Modifications.
Cystic Fibrosis
Trafficking of GFP-tagged DeltaF508-CFTR to the plasma membrane in a polarized epithelial cell line.
Cystic Fibrosis
Trans-complementation of E1-deleted adenovirus: a new vector to reduce the possibility of codissemination of wild-type and recombinant adenoviruses.
Cystic Fibrosis
Transcript analysis of CFTR frameshift mutations in lymphocytes using the reverse transcription-polymerase chain reaction technique and the protein truncation test.
Cystic Fibrosis
Transcription of cystic fibrosis transmembrane conductance regulator requires a CCAAT-like element for both basal and cAMP-mediated regulation.
Cystic Fibrosis
Transcriptional adaptation to cystic fibrosis transmembrane conductance regulator deficiency.
Cystic Fibrosis
Transcriptional analysis of cystic fibrosis airways at single-cell resolution reveals altered epithelial cell states and composition.
Cystic Fibrosis
Transcriptional repression of the cystic fibrosis transmembrane conductance regulator gene, mediated by CCAAT displacement protein/cut homolog, is associated with histone deacetylation.
Cystic Fibrosis
Transcriptional targeting in the airway using novel gene regulatory elements.
Cystic Fibrosis
Transcriptome Profiling and Molecular Therapeutic Advances in Cystic Fibrosis: Recent Insights.
Cystic Fibrosis
Transcriptomic analysis of CFTR-impaired endothelial cells reveals a pro-inflammatory phenotype.
Cystic Fibrosis
Transcriptomic responses to Ivacaftor and prediction of Ivacaftor clinical responsiveness.
Cystic Fibrosis
Transcytosis maintains CFTR apical polarity in the face of constitutive and mutation-induced basolateral missorting.
Cystic Fibrosis
Transduction of Surface and Basal Cells in Rhesus Macaque Lung Following Repeat Dosing with AAV1CFTR.
Cystic Fibrosis
Transepithelial ion transport is suppressed in hypoxic sinonasal epithelium.
Cystic Fibrosis
Transfected beta3- but not beta2-adrenergic receptors regulate cystic fibrosis transmembrane conductance regulator activity via a new pathway involving the mitogen-activated protein kinases extracellular signal-regulated kinases.
Cystic Fibrosis
Transfection of wild-type CFTR into cystic fibrosis lymphocytes restores chloride conductance at G1 of the cell cycle.
Cystic Fibrosis
Transfer of a constitutive viral promoter-cystic fibrosis transmembrane conductance regulator cDNA to human epithelial cells conveys resistance to down-regulation of cAMP-regulated Cl- secretion in the presence of inflammatory stimuli.
Cystic Fibrosis
Transfer of the Cystic Fibrosis Transmembrane Conductance Regulator to Human Cystic Fibrosis Cells Mediated by Extracellular Vesicles.
Cystic Fibrosis
Transforming growth factor beta1 impairs CFTR-mediated anion secretion across cultured porcine vas deferens epithelial monolayer via the p38 MAPK pathway.
Cystic Fibrosis
Transforming growth factor beta1 inhibits cystic fibrosis transmembrane conductance regulator-dependent cAMP-stimulated alveolar epithelial fluid transport via a phosphatidylinositol 3-kinase-dependent mechanism.
Cystic Fibrosis
Transforming Growth Factor-?1 Selectively Recruits microRNAs to the RNA-Induced Silencing Complex and Degrades CFTR mRNA under Permissive Conditions in Human Bronchial Epithelial Cells.
Cystic Fibrosis
Transient anchorage of cross-linked glycosyl-phosphatidylinositol-anchored proteins depends on cholesterol, Src family kinases, caveolin, and phosphoinositides.
Cystic Fibrosis
Transient gene expression from yeast artificial chromosome DNA in mammalian cells is enhanced by adenovirus.
Cystic Fibrosis
Transient in utero disruption of cystic fibrosis transmembrane conductance regulator causes phenotypic changes in alveolar type II cells in adult rats.
Cystic Fibrosis
Transient transfection of polarized epithelial monolayers with CFTR and reporter genes using efficacious lipids.
Cystic Fibrosis
Transimmortalized mouse intestinal cells (m-ICc12) that maintain a crypt phenotype.
Cystic Fibrosis
Transmembrane domain of cystic fibrosis transmembrane conductance regulator: design, characterization, and secondary structure of synthetic peptides m1-m6.
Cystic Fibrosis
TRANSMEMBRANE PROTEIN 16A (TMEM16A) IS A CA++ REGULATED CL- SECRETORY CHANNEL IN MOUSE AIRWAYS.
Cystic Fibrosis
Transmembrane transport of endo- and xenobiotics by mammalian ATP-binding cassette multidrug resistance proteins.
Cystic Fibrosis
Transmission of male infertility and intracytoplasmic sperm injection (mini-review).
Cystic Fibrosis
Transmission ratio distortion and maternal effects confound the analysis of modulators of cystic fibrosis disease severity on 19q13.
Cystic Fibrosis
Transport rates of GABA transporters: regulation by the N-terminal domain and syntaxin 1A.
Cystic Fibrosis
Treating the Airway Consequences of Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction.
Cystic Fibrosis
Treating the Underlying Cystic Fibrosis Transmembrane Conductance Regulator Defect in Patients with Cystic Fibrosis.
Cystic Fibrosis
Treatment of Cystic Fibrosis Patients Homozygous for F508del with Lumacaftor-Ivacaftor (Orkambi®) Restores Defective CFTR Channel Function in Circulating Mononuclear Cells.
Cystic Fibrosis
Treatment of human airway epithelial Calu-3 cells with a peptide-nucleic acid (PNA) targeting the microRNA miR-101-3p is associated with increased expression of the cystic fibrosis Transmembrane Conductance Regulator () gene.
Cystic Fibrosis
Treatment Options for cystic fibrosis: State of the Art and Future Perspectives.
Cystic Fibrosis
Trichomonas vaginalis infection impairs anion secretion in vaginal epithelium.
Cystic Fibrosis
TRIM44 links the UPS to SQSTM1/p62-dependent aggrephagy and removing misfolded proteins.
Cystic Fibrosis
Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells.
Cystic Fibrosis
Trimethylangelicin reduces IL-8 transcription and potentiates CFTR function.
Cystic Fibrosis
Triple combination cystic fibrosis transmembrane conductance regulator modulator therapy in the real world - opportunities and challenges.
Cystic Fibrosis
Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes.
Cystic Fibrosis
Triplet CFTR modulators: future prospects for treatment of cystic fibrosis.
Cystic Fibrosis
TRPC6 channel translocation into phagosomal membrane augments phagosomal function.
Cystic Fibrosis
TRPC6 Links Ca2+ Mishandling to CFTR Channel Dysfunction in Cystic Fibrosis.
Cystic Fibrosis
Truncating Mutations in the Adhesion G Protein-Coupled Receptor G2 Gene ADGRG2 Cause an X-Linked Congenital Bilateral Absence of Vas Deferens.
Cystic Fibrosis
Tumor necrosis factor modulation of expression of the cystic fibrosis transmembrane conductance regulator gene.
Cystic Fibrosis
Tumor necrosis factor-?-mediated downregulation of the cystic fibrosis transmembrane conductance regulator drives pathological sphingosine-1-phosphate signaling in a mouse model of heart failure.
Cystic Fibrosis
Tuning of CFTR chloride channel function by location of positive charges within the pore.
Cystic Fibrosis
Turnover of the cystic fibrosis transmembrane conductance regulator (CFTR): slow degradation of wild-type and delta F508 CFTR in surface membrane preparations of immortalized airway epithelial cells.
Cystic Fibrosis
Two CFTR mutations within codon 970 differently impact on the chloride channel functionality.
Cystic Fibrosis
Two cystic fibrosis transmembrane conductance regulator mutations have different effects on both pulmonary phenotype and regulation of outwardly rectified chloride currents.
Cystic Fibrosis
Two mechanisms of genistein inhibition of cystic fibrosis transmembrane conductance regulator Cl- channels expressed in murine cell line.
Cystic Fibrosis
Two mild cystic fibrosis-associated mutations result in severe cystic fibrosis when combined in cis and reveal a residue important for cystic fibrosis transmembrane conductance regulator processing and function.
Cystic Fibrosis
Two new mutations detected by single-strand conformation polymorphism analysis in cystic fibrosis from Russia.
Cystic Fibrosis
Two novel and correlated CF-causing insertions in the (TG)mTn tract of the CFTR gene.
Cystic Fibrosis
Two novel frame-shift mutations: 977 insA in exon 6B, and 4016 insT in exon 21, of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
Two salt bridges differentially contribute to the maintenance of cystic fibrosis transmembrane conductance regulator (CFTR) channel function.
Cystic Fibrosis
Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.
Cystic Fibrosis
Two years of newborn screening for cystic fibrosis in Turkey: Çukurova experience.
Cystic Fibrosis
Two-pore-domain potassium channels support anion secretion from human airway Calu-3 epithelial cells.
Cystic Fibrosis
Type I, II, III, IV, and V cystic fibrosis transmembrane conductance regulator defects and opportunities for therapy.
Cystic Fibrosis
Type II protein kinase A regulates CFTR in airway, pancreatic, and intestinal cells.
Cystic Fibrosis
Type IV(B) pili are required for invasion but not for adhesion of Salmonella enterica serovar Typhi into BHK epithelial cells in a cystic fibrosis transmembrane conductance regulator-independent manner.
Cystic Fibrosis
Tyrosine kinase c-Src constitutes a bridge between cystic fibrosis transmembrane regulator channel failure and MUC1 overexpression in cystic fibrosis.
Cystic Fibrosis
Tyrosine kinase-independent extracellular action of genistein on the CFTR Cl- channel in guinea pig ventricular myocytes and CFTR-transfected mouse fibroblasts.
Cystic Fibrosis
Tyrosine phosphorylation is a novel pathway for regulation of chloride secretion in shark rectal gland.
Cystic Fibrosis
Ubiquitin C-terminal hydrolase-L1 protects cystic fibrosis transmembrane conductance regulator from early stages of proteasomal degradation.
Cystic Fibrosis
Ubiquitin-mediated proteasomal degradation of ABC transporters: a new aspect of genetic polymorphisms and clinical impacts.
Cystic Fibrosis
Ubiquitination and degradation of CFTR by the E3 ubiquitin ligase MARCH2 through its association with adaptor proteins CAL and STX6.
Cystic Fibrosis
Ubiquitination of disease-causing CFTR variants in a microsome-based assay.
Cystic Fibrosis
UBXD1 is a VCP-interacting protein that is involved in ER-associated degradation.
Cystic Fibrosis
Ultrastructural lesions in the small bowel of patients with cystic fibrosis.
Cystic Fibrosis
Ultrastructural localization of variant forms of cystic fibrosis transmembrane conductance regulator in human bronchial epithelial of xenografts.
Cystic Fibrosis
Understanding how cystic fibrosis mutations cause a loss of Cl- channel function.
Cystic Fibrosis
Understanding how cystic fibrosis mutations disrupt CFTR function: From single molecules to animal models.
Cystic Fibrosis
Understanding human disease mutations through the use of interspecific genetic variation.
Cystic Fibrosis
Understanding protein kinase CK2 mis-regulation upon F508del CFTR expression.
Cystic Fibrosis
Understanding the population structure of North American patients with cystic fibrosis.
Cystic Fibrosis
Unexpected inactivation of acceptor consensus splice sequence by a -3 C to T transition in intron 2 of the CFTR gene.
Cystic Fibrosis
Unique microbial communities persist in individual cystic fibrosis patients throughout a clinical exacerbation.
Cystic Fibrosis
Unique mutations of the cystic fibrosis transmembrane conductance regulator gene of three cases of cystic fibrosis in Nagasaki, Japan.
Cystic Fibrosis
Unique presentations and chronic complications in adult cystic fibrosis: do they teach us anything about CFTR?
Cystic Fibrosis
Unitary chloride channels activated by protein kinase C in guinea pig ventricular myocytes.
Cystic Fibrosis
Unplugging Mucus in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Cystic Fibrosis
Unraveling monogenic channelopathies and their implications for complex polygenic disease.
Cystic Fibrosis
Unraveling the complex genetic model for cystic fibrosis: pleiotropic effects of modifier genes on early cystic fibrosis-related morbidities.
Cystic Fibrosis
Unravelling the Regions of Mutant F508del-CFTR More Susceptible to the Action of Four Cystic Fibrosis Correctors.
Cystic Fibrosis
Unusual Cystic Fibrosis Transmembrane Conductance Regulator Mutations and Liver Disease: A Case Series and Review of the Literature.
Cystic Fibrosis
Unveiling the degradative route of the V247M ?-sarcoglycan mutant responsible for LGMD-2D.
Cystic Fibrosis
Up-regulated cystic fibrosis transmembrane conductance regulator after anal stenosis in rats.
Cystic Fibrosis
Up-regulation of acid-gated Na(+) channels (ASICs) by cystic fibrosis transmembrane conductance regulator co-expression in Xenopus oocytes.
Cystic Fibrosis
Up-regulation of AMP-activated Kinase by Dysfunctional Cystic Fibrosis Transmembrane Conductance Regulator in Cystic Fibrosis Airway Epithelial Cells Mitigates Excessive Inflammation.
Cystic Fibrosis
Update on clinical trials in the treatment of pulmonary disease in patients with cystic fibrosis.
Cystic Fibrosis
Update on Respiratory Fungal Infections in Cystic Fibrosis Lung Disease and after Lung Transplantation.
Cystic Fibrosis
Update on the cellular and molecular aspects of cystic fibrosis transmembrane conductance regulator (CFTR) and male fertility.
Cystic Fibrosis
Upregulation of CFTR expression but not SLC26A3 and SLC9A3 in ulcerative colitis.
Cystic Fibrosis
Upregulation of CFTR in patients with endometriosis and its involvement in NF?B-uPAR dependent cell migration.
Cystic Fibrosis
Upregulation of CFTR Protects against Palmitate-Induced Endothelial Dysfunction by Enhancing Autophagic Flux.
Cystic Fibrosis
Upregulation of cystic fibrosis transmembrane conductance regulator expression by oestrogen and Bak Foong Pill in mouse uteri.
Cystic Fibrosis
Uptake of fluorescent dyes associated with the functional expression of the cystic fibrosis transmembrane conductance regulator in epithelial cells.
Cystic Fibrosis
Urinary Exosomes of Patients with Cystic Fibrosis Unravel CFTR-Related Renal Disease.
Cystic Fibrosis
Uroguanylin and guanylate cyclase C in the human pancreas: expression and mutuality of ligand/receptor localization as indicators of intercellular paracrine signaling pathways.
Cystic Fibrosis
Ursodeoxycholate modulates bile flow and bile salt pool independently from the cystic fibrosis transmembrane regulator (Cftr)in mice.
Cystic Fibrosis
Ursodeoxycholic acid inhibits ENaC and Na/K pump activity to restore airway surface liquid height in cystic fibrosis bronchial epithelial cells.
Cystic Fibrosis
Use of a High-Throughput Phenotypic Screening Strategy to Identify Amplifiers, a Novel Pharmacological Class of Small Molecules That Exhibit Functional Synergy with Potentiators and Correctors.
Cystic Fibrosis
Use of a membrane potential-sensitive probe to assess biological expression of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Use of nasal potential difference and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis.
Cystic Fibrosis
Use of sodium butyrate to enhance production of retroviral vectors expressing CFTR cDNA.
Cystic Fibrosis
Utilization of Liquid Chromatography Mass Spectrometry Analyses to Identify LKB1-APC Interaction in Modulating Wnt/?-Catenin Pathway of Lung Cancer Cells.
Cystic Fibrosis
Validation of double gradient denaturing gradient gel electrophoresis through multigenic retrospective analysis.
Cystic Fibrosis
Validation of high-resolution DNA melting analysis for mutation scanning of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Cystic Fibrosis
Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis.
Cystic Fibrosis
Validity and Reliability of a Novel Multimodal Questionnaire for the Assessment of Abdominal Symptoms in People with Cystic Fibrosis (CFAbd-Score).
Cystic Fibrosis
VAMP-associated Proteins (VAP) as Receptors That Couple Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Proteostasis with Lipid Homeostasis.
Cystic Fibrosis
Vardenafil increases intracellular accumulation of the most prevalent mutant cystic fibrosis transmembrane conductance regulator (CTFR) in human bronchial epithelial cells.
Cystic Fibrosis
Variable deletion of exon 9 coding sequences in cystic fibrosis transmembrane conductance regulator gene mRNA transcripts in normal bronchial epithelium.
Cystic Fibrosis
Variable levels of normal RNA in different fetal organs carrying a cystic fibrosis transmembrane conductance regulator splicing mutation.
Cystic Fibrosis
Variable penetrance and expressivity of the splice altering 5T sequence in the cystic fibrosis gene.
Cystic Fibrosis
Variable reactivity of an engineered cysteine at position 338 in cystic fibrosis transmembrane conductance regulator reflects different chemical states of the thiol.
Cystic Fibrosis
Variable Responses to CFTR Correctors in vitro: Estimating the Design Effect in Precision Medicine.
Cystic Fibrosis
Variants in CFTR untranslated regions are associated with congenital bilateral absence of the vas deferens.
Cystic Fibrosis
Variants in mannose-binding lectin and tumour necrosis factor alpha affect survival in cystic fibrosis.
Cystic Fibrosis
Variants in the glutamate-cysteine-ligase gene are associated with cystic fibrosis lung disease.
Cystic Fibrosis
Variants in the interleukin 8 gene and the response to inhaled bronchodilators in cystic fibrosis.
Cystic Fibrosis
Variation in a repeat sequence determines whether a common variant of the cystic fibrosis transmembrane conductance regulator gene is pathogenic or benign.
Cystic Fibrosis
Variation in branchial expression among insulin-like growth-factor binding proteins (igfbps) during Atlantic salmon smoltification and seawater exposure.
Cystic Fibrosis
Variations in the expression of vasotocin and isotocin receptor genes in the gilthead sea bream Sparus aurata during different osmotic challenges.
Cystic Fibrosis
Varied clinical significance of ATP-binding cassette C sub-family members for lung adenocarcinoma.
Cystic Fibrosis
Vasoactive Intestinal Peptide Derived From Liver Mesenchymal Cells Mediates Tight Junction Assembly in Mouse Intrahepatic Bile Ducts.
Cystic Fibrosis
Vasoactive intestinal peptide increases cystic fibrosis transmembrane conductance regulator levels in the apical membrane of Calu-3 cells through a protein kinase C-dependent mechanism.
Cystic Fibrosis
Vasoactive intestinal peptide regulates sinonasal mucociliary clearance and synergizes with histamine in stimulating sinonasal fluid secretion.
Cystic Fibrosis
Vasoactive intestinal peptide, forskolin, and genistein increase apical CFTR trafficking in the rectal gland of the spiny dogfish, Squalus acanthias. Acute regulation of CFTR trafficking in an intact epithelium.
Cystic Fibrosis
Vasopressin and cAMP stimulate electrogenic chloride secretion in an IMCD cell line.
Cystic Fibrosis
Vasopressin regulates the phosphorylation state of AMP-activated protein kinase (AMPK) in MDCK-C7 cells.
Cystic Fibrosis
Vasopressin stimulates long-term net chloride secretion in cortical collecting duct cells.
Cystic Fibrosis
Vasopressin-stimulated chloride transport in transimmortalized mouse cell lines derived from the distal convoluted tubule and cortical and inner medullary collecting ducts.
Cystic Fibrosis
VCP/p97 AAA-ATPase does not interact with the endogenous wild-type cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Vector-specific complementation profiles of two independent primary defects in cystic fibrosis airways.
Cystic Fibrosis
Vesicular targeting and the control of ion secretion in epithelial cells: implications for cystic fibrosis.
Cystic Fibrosis
Vibrio parahaemolyticus induces inflammation-associated fluid accumulation via activation of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
VIP regulates CFTR membrane expression and function in Calu-3 cells by increasing its interaction with NHERF1 and P-ERM in a VPAC1- and PKC?-dependent manner.
Cystic Fibrosis
Visualization of oligonucleotide probes and point mutations in interphase nuclei and DNA fibers using rolling circle DNA amplification.
Cystic Fibrosis
Visualization of single proteins from stripped native cell membranes: a protocol for high-resolution atomic force microscopy.
Cystic Fibrosis
Vitamin C controls the cystic fibrosis transmembrane conductance regulator chloride channel.
Cystic Fibrosis
Voltage-dependent block of the cystic fibrosis transmembrane conductance regulator Cl- channel by two closely related arylaminobenzoates.
Cystic Fibrosis
Voltage-dependent calcium and chloride currents in S17 bone marrow stromal cell line.
Cystic Fibrosis
Voltage-dependent flickery block of an open cystic fibrosis transmembrane conductance regulator (CFTR) channel pore.
Cystic Fibrosis
Voltage-dependent gating of the cystic fibrosis transmembrane conductance regulator Cl- channel.
Cystic Fibrosis
Voltage-sensitive gating induced by a mutation in the fifth transmembrane domain of CFTR.
Cystic Fibrosis
Volume-activated chloride current is not related to P-glycoprotein overexpression.
Cystic Fibrosis
Volume-regulated chloride channels associated with the human multidrug-resistance P-glycoprotein.
Cystic Fibrosis
VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
Cystic Fibrosis
VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
Cystic Fibrosis
Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle.
Cystic Fibrosis
VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1.
Cystic Fibrosis
Walker mutations reveal loose relationship between catalytic and channel-gating activities of purified CFTR (cystic fibrosis transmembrane conductance regulator).
Cystic Fibrosis
What is the role of cystic fibrosis transmembrane conductance regulator dysfunction in primary sclerosing cholangitis?
Cystic Fibrosis
What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?
Cystic Fibrosis
What shall we do with the damaged proteins in lung disease? Ask the proteasome!
Cystic Fibrosis
What we know and what we do not know about cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Wheat bran components modulate intestinal bacteria and gene expression of barrier function relevant proteins in a piglet model.
Cystic Fibrosis
Whole cell Cl- conductances in mouse choroid plexus epithelial cells do not require CFTR expression.
Cystic Fibrosis
Whole-blood transcriptomic responses to lumacaftor/ivacaftor therapy in cystic fibrosis.
Cystic Fibrosis
Whole-Exome Sequencing Identified CFTR Variants in Two Consanguineous Families in China.
Cystic Fibrosis
Why Mouse Airway Submucosal Gland Serous Cells Do Not Secrete Fluid in Response to cAMP Stimulation.
Cystic Fibrosis
Widespread airway distribution and short-term phenotypic correction of cystic fibrosis pigs following aerosol delivery of piggyBac/adenovirus.
Cystic Fibrosis
Widespread and efficient marker gene expression in the airway epithelia of fetal sheep after minimally invasive tracheal application of recombinant adenovirus in utero.
Cystic Fibrosis
WNK1 and p38-MAPK distribution in ionocytes and accessory cells of euryhaline teleost fish implies ionoregulatory function.
Cystic Fibrosis
X chromosome transmission ratio distortion in Cftr +/- intercross-derived mice.
Cystic Fibrosis
X-ray microanalysis of apical fluid in cystic fibrosis airway epithelial cell lines.
Cystic Fibrosis
X-ray microanalysis of cell elements in normal and cystic fibrosis jejunum: evidence for chloride secretion in villi.
Cystic Fibrosis
XV-2c/KM19 haplotypes analysis of cystic fibrosis patients from western Mexico.
Cystic Fibrosis
Yes-associated protein 65 localizes p62(c-Yes) to the apical compartment of airway epithelia by association with EBP50.
Cystic Fibrosis
Yet another role for the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
Zinc Deficiency via a Splice Switch in Zinc Importer ZIP2/SLC39A2 Causes Cystic Fibrosis-Associated MUC5AC Hypersecretion in Airway Epithelial Cells.
Cystic Fibrosis
[(Dihydroindenyl)oxy]alkonic acid inhibits the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis
[3120+1kbdel8.6kb]+[p.N1303K] genotype in an Emirati cystic fibrosis patient: indication of a founder mutation in Palestinian Arabs.
Cystic Fibrosis
[A case report of cystic fibrosis and review of 16 cases of cystic fibrosis in Chinese patients]
Cystic Fibrosis
[Aerosol administration of a replication defective recombinant adenovirus expressing normal human cDNA-CFTR in the respiratory tractus in patients with cystic fibrosis]
Cystic Fibrosis
[Analysis of the spectra of mutations and polymorphic loci of cystic fibrosis transmembrane conductance regulator in the population of Bashkortostan]
Cystic Fibrosis
[Antisense oligonucleotides for therapy of cystic fibrosis : Inhibition of sodium absorption mediated by ENaC in nasal epithelial cells.]
Cystic Fibrosis
[Association of CFTR gene polymorphism with congenital bilateral absence of vas deferens in ethnic Han Chinese patients].
Cystic Fibrosis
[Benzamil and mucoviscidosis. Primary culture of nasal mucosa as an electrophysiologic in vitro model]
Cystic Fibrosis
[Butyl-p-hydroxybenzoate stimulates cystic fibrosis transmembrane conductance regulator Cl- transport]
Cystic Fibrosis
[CF Lung Disease - a German S3 Guideline: Module 2: Diagnostics and Treatment in Chronic Infection with Pseudomonas aeruginosa].
Cystic Fibrosis
[CFTR as cAMP-dependent chloride channels and as cAMP-dependent regulator of sodium channels]
Cystic Fibrosis
[CFTR gene analyis in 207 patients with cystic fibrosis in southwest France: high frequency of N1303K and 1811+1.6bA>G mutations]
Cystic Fibrosis
[CFTR protein and molecular mechanisms of pulmonary involvement in cystic fibrosis]
Cystic Fibrosis
[Clinical and molecular genetic study of cystic fibrosis in the 5th Region of Chile]
Cystic Fibrosis
[Clinical aspects and genetic specificities of cystic fibrosis in Reunion Island]
Cystic Fibrosis
[Comparisons of pulmonary and sinonasal lesions in patients with cystic fibrosis. Evaluation using computerized tomography]
Cystic Fibrosis
[Correlation between CFTR 5T polymorphisms and the risk of congenital bilateral absence of the vas deferens].
Cystic Fibrosis
[Correlation between phenotype and genotype in a group of patients with cystic fibrosis]
Cystic Fibrosis
[Cystic fibrosis gene mutations in the West of France: clinical application]
Cystic Fibrosis
[Cystic fibrosis transmembrane conductance regulator (CFTR) gene: mutations and clinical phenotypes]
Cystic Fibrosis
[Detection of deltaF508 deletion causing cystic fibrosis, using quantitative real-time PCR]
Cystic Fibrosis
[Detection of mutations in the CFTR gene using the PCR-dependent preferential homoduplex formation assay (PCR-PHFA) system]
Cystic Fibrosis
[Detection of the mutation of all the exons of the CFTR gene in Chinese men with congenital bilateral absence of the vas deferens].
Cystic Fibrosis
[Effect of Electroacupuncture Stimulation of "Neiguan" (PC 6) on Expression of Myocardial Chloride Channel-related Genes, PKC and PKG Proteins in Myocardial Ischemia Rats].
Cystic Fibrosis
[Effects of intratympanic injection of dexamethasone on endolymphatic hydrops and changes in guinea pigs inner ear CFTR expression].
Cystic Fibrosis
[Effects of monocarboxylic acid derivatives on cardiac ventricular CFTR Cl- channels in guinea pig]
Cystic Fibrosis
[Epidemiologic study of the cystic fibrosis gene in the Champagne-Ardenne region]
Cystic Fibrosis
[Establishment and evaluation of methods for determinating cystic fibrosis transmembrane conductance regulator quantitatively].
Cystic Fibrosis
[Expression of cystic fibrosis transmembrane conductance regulator in human endometrium]
Cystic Fibrosis
[Expressions of cystic fibrosis transmembrane conductance regulator in epithelial cells of nasal polyps and normal turbinate mucosae]
Cystic Fibrosis
[Frequency of delta F508 mutation in Venezuelan patients with cystic fibrosis]
Cystic Fibrosis
[From gene to disease; from defective chloride ion transport to cystic fibrosis]
Cystic Fibrosis
[Functional compartmentation of the endocrine action of cardiac natriuretic peptides]
Cystic Fibrosis
[Genetic mutations as a cause of acute recurrent pancreatitis in children - case report and literature review].
Cystic Fibrosis
[Genotypes of cystic fibrosis (CF) reported in the world and polymorphisms of cystic fibrosis transmembrane conductance regulator (CFTR) gene in Japanese]
Cystic Fibrosis
[Identification of mutation in the gene cystic fibrosis transmembrane regulator (CFTR) in Chilean patients with cystic fibrosis]
Cystic Fibrosis
[Impact of Cystic Fibrosis Transmembrane Conductance Regulator on Malignant? Properties of KRAS Mutant Lung Adenocarcinoma A549 Cells].
Cystic Fibrosis
[Impact of the CFTR chloride channel on the cytoskeleton of mouse Sertoli cells].
Cystic Fibrosis
[Inhibitors of intra-cystic secretion in autosomal dominant polycystic kidney disease (ADPKD): a possible way forward].
Cystic Fibrosis
[Ion transport in nasal and paranasal sinus mucosa in mucoviscidosis and chronic sinusitis]
Cystic Fibrosis
[MDR (multidrug resistance) in hepatocarcinoma clinical-therapeutic implications]
Cystic Fibrosis
[Microbiological diagnosis of bronchopulmonary colonization-infection in cystic fibrosis.]
Cystic Fibrosis
[Molecular diagnosis of cystic fibrosis in 93 Argentinean patients and detection of heterozygotes in affected families. Impact on health services and therapeutic advances]
Cystic Fibrosis
[Molecular genetic analysis of TUB18 and TUB20 intragenic polymorphism and various mutations of the CFTR gene in the Moscow region]
Cystic Fibrosis
[Molecular genetics principles in cystic fibrosis. An example of genetic illness in pneumology]
Cystic Fibrosis
[Mucoviscidosis: Background, risk factors, diagnosis strategy, treatment principles]
Cystic Fibrosis
[Mutation in the cystic fibrosis transmembrane-regulator gene in bilateral congenital ductus deferens aplasia]
Cystic Fibrosis
[Pancreatitis in cystic fibrosis: association with genotype and pancreatic status.]
Cystic Fibrosis
[Patients with cystic fibrosis become adults : Treatment hopes and disappointments].
Cystic Fibrosis
[PMP70, the 70-kDa peroxisomal membrane protein: a member of the ATP-binding cassette transporters]
Cystic Fibrosis
[Polymethoxylated flavonoids activate cystic fibrosis transmembrane conductance regulator chloride channel].
Cystic Fibrosis
[Possibilities and prospects of the use of DNA analysis in the diagnosis and prevention of inherited disease in the Ukraine]
Cystic Fibrosis
[Post-translational ligation and function of dual-vector transferred split CFTR gene].
Cystic Fibrosis
[Post-translational ligation of split CFTR severed before TMD2 and its chloride channel function].
Cystic Fibrosis
[Problems of gene transfer to human airway in the patients with respiratory diseases]
Cystic Fibrosis
[Protective effect of Yishen Tongluo Recipe against benzo(a)pyrene-induced sperm DNA methylation changes in male rats].
Cystic Fibrosis
[Real-world effectiveness of ivacaftor in children with cystic fibrosis and the G551D mutation].
Cystic Fibrosis
[Regulation by vasopressin of NaCl absorption in the renal collecting duct]
Cystic Fibrosis
[Relationship of Cystic Fibrosis Transmembrane Conductance Regulator Expression with Clinical Features and Prognosis in Patients with Acute Leukemia].
Cystic Fibrosis
[Screening of the delta-F508 mutation and analysis of two single nucleotide polymorphism of the CFTR gene, in a sample of the general population of ValparaÃso, Chile.]
Cystic Fibrosis
[Severe male infertility. Genetic investigation and counseling prior to intracytoplasmic sperm injection]
Cystic Fibrosis
[The activation effect of nobiletin on cystic fibrosis transmembrane conductance regulator chloride channel].
Cystic Fibrosis
{alpha}AP2 directs myosin VI-dependent endocytosis of cystic fibrosis transmembrane conductance regulator chloride channels in the intestine.
Cysts
A chalcone derivative retards renal cyst enlargement by inhibiting fluid secretion and cell proliferation in an in vitro model of polycystic kidney disease.
Cysts
A potential strategy for reducing cysts in autosomal dominant polycystic kidney disease with a CFTR corrector.
Cysts
A regulatory role of polycystin-1 on cystic fibrosis transmembrane conductance regulator plasma membrane expression.
Cysts
Activation of AMP-activated kinase as a strategy for managing autosomal dominant polycystic kidney disease.
Cysts
Anoctamin 1 induces calcium-activated chloride secretion and proliferation of renal cyst-forming epithelial cells.
Cysts
cAMP regulates cell proliferation and cyst formation in autosomal polycystic kidney disease cells.
Cysts
Cyst growth in ADPKD is prevented by pharmacological and genetic inhibition of TMEM16A in vivo.
Cysts
Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth.
Cysts
Inhibition of histone deacetylase 6 activity reduces cyst growth in polycystic kidney disease.
Cysts
KCa3.1 potassium channels are critical for cAMP-dependent chloride secretion and cyst growth in autosomal-dominant polycystic kidney disease.
Cysts
Nanomolar Potency Pyrimido-pyrrolo-quinoxalinedione CFTR Inhibitor Reduces Cyst Size in a Polycystic Kidney Disease Model.
Cysts
Pharmacological reversal of renal cysts from secretion to absorption suggests a potential therapeutic strategy for managing autosomal dominant polycystic kidney disease.
Cysts
Steviol Reduces MDCK Cyst Formation and Growth by Inhibiting CFTR Channel Activity and Promoting Proteasome-Mediated CFTR Degradation.
Cysts
Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease.
Cysts
The relationship between cell proliferation, Cl- secretion, and renal cyst growth: a study using CFTR inhibitors.
Cysts
The zebrafish Kupffer's vesicle as a model system for the molecular mechanisms by which the lack of Polycystin-2 leads to stimulation of CFTR.
Cysts
Therapeutic potential of cystic fibrosis transmembrane conductance regulator (CFTR) inhibitors in polycystic kidney disease.
Cysts
Vasoactive Intestinal Peptide Derived From Liver Mesenchymal Cells Mediates Tight Junction Assembly in Mouse Intrahepatic Bile Ducts.
Dacryocystitis
Changes of chloride channels in the lacrimal glands of a rabbit model of Sjögren syndrome.
Dehydration
A specific cystic fibrosis mutation (T3381) associated with the phenotype of isolated hypotonic dehydration.
Dehydration
Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies.
Dehydration
Auto-obliteration of maxillary sinuses through osteoneogenesis in children with cystic fibrosis: A possible new way to reduce morbidity.
Dehydration
Cigarette Smoke-induced Ca2+ Release Leads to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Dysfunction.
Dehydration
Physiological impact of abnormal lipoxin A? production on cystic fibrosis airway epithelium and therapeutic potential.
Dehydration
Reduced NHE3-mediated Na+ absorption increases survival and decreases the incidence of intestinal obstructions in cystic fibrosis mice.
Dehydration
Rheological effects of hypertonic saline and sodium bicarbonate solutions on cystic fibrosis sputum in vitro.
Dehydration
Roflumilast combined with adenosine increases mucosal hydration in human airway epithelial cultures after cigarette smoke exposure.
Dehydration
Ursodeoxycholic acid inhibits ENaC and Na/K pump activity to restore airway surface liquid height in cystic fibrosis bronchial epithelial cells.
Digestive System Diseases
Correction of CFTR malfunction and stimulation of Ca-activated Cl channels restore HCO3- secretion in cystic fibrosis bile ductular cells.
Drug-Related Side Effects and Adverse Reactions
Innate immunity and chronic rhinosinusitis: What we have learned from animal models.
Dry Eye Syndromes
Nanomolar-Potency Aminophenyl-1,3,5-triazine Activators of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Chloride Channel for Prosecretory Therapy of Dry Eye Diseases.
Dry Eye Syndromes
Pro-Secretory Activity and Pharmacology in Rabbits of an Aminophenyl-1,3,5-Triazine CFTR Activator for Dry Eye Disorders.
Dyspnea
Assessing effects of inhaled antibiotics in adults with non-cystic fibrosis bronchiectasis--experiences from recent clinical trials.
Dyspnea
Unique mutations of the cystic fibrosis transmembrane conductance regulator gene of three cases of cystic fibrosis in Nagasaki, Japan.
Echogenic Bowel
Calculating posterior cystic fibrosis risk with echogenic bowel and one characterized cystic fibrosis mutation: avoiding pitfalls in the risk calculations.
Echogenic Bowel
Mid-trimester hyperechogenic bowel in a fetus of Japanese origin carrying a new mutation of CFTR gene (L548Q).
Embryo Loss
CFTR Depletion Confers Hypersusceptibility to Mycobacterium fortuitum in a Zebrafish Model.
Endometrial Neoplasms
Lower Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Promotes the Proliferation and Migration of Endometrial Carcinoma.
Esophageal Neoplasms
CFTR inhibits the invasion and growth of esophageal cancer cells by inhibiting the expression of NF-?B.
Esophageal Squamous Cell Carcinoma
Expression and Role of CFTR in Human Esophageal Squamous Cell Carcinoma.
Exocrine Pancreatic Insufficiency
'CFTR-opathies': disease phenotypes associated with cystic fibrosis transmembrane regulator gene mutations.
Exocrine Pancreatic Insufficiency
A proinflammatory, antiapoptotic phenotype underlies the susceptibility to acute pancreatitis in cystic fibrosis transmembrane regulator (-/-) mice.
Exocrine Pancreatic Insufficiency
Accumulation of ceramide in the trachea and intestine of cystic fibrosis mice causes inflammation and cell death.
Exocrine Pancreatic Insufficiency
CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis.
Exocrine Pancreatic Insufficiency
Characterization of two distinct chloride channels in cultured dog pancreatic duct epithelial cells.
Exocrine Pancreatic Insufficiency
Cystic fibrosis mutation classes in pediatric otitis media - Fickle or faulty?
Exocrine Pancreatic Insufficiency
Heterogeneity of phenotype in two cystic fibrosis patients homozygous for the CFTR exon 11 mutation G551D.
Exocrine Pancreatic Insufficiency
Homozygous (TG)11 allele in intron 8 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene has a protective role against bicarbonate decrease in pure pancreatic juice among Japanese male alcoholics.
Exocrine Pancreatic Insufficiency
Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report.
Exocrine Pancreatic Insufficiency
Liver cirrhosis and portal hypertension in cystic fibrosis.
Exocrine Pancreatic Insufficiency
Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis.
Exocrine Pancreatic Insufficiency
Pancreatic complications in children with cystic fibrosis.
Exocrine Pancreatic Insufficiency
Pancreatitis in A Patient with Cystic Fibrosis Taking Ivacaftor.
Exocrine Pancreatic Insufficiency
Phenotype of CF and the effects of possible modifier genes.
Exocrine Pancreatic Insufficiency
Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis.
Eye Diseases
Pro-Secretory Activity and Pharmacology in Rabbits of an Aminophenyl-1,3,5-Triazine CFTR Activator for Dry Eye Disorders.
Fecal Impaction
Up-regulated cystic fibrosis transmembrane conductance regulator after anal stenosis in rats.
Funnel Chest
Pulmonary nontuberculous mycobacterial disease: prospective study of a distinct preexisting syndrome.
Gastrointestinal Diseases
Plasma membrane-localized TMEM16 proteins are indispensable for expression of CFTR.
Gastrointestinal Neoplasms
Emerging role of cystic fibrosis transmembrane conductance regulator - an epithelial chloride channel in gastrointestinal cancers.
Genetic Diseases, Inborn
A chimeric type 2 adenovirus vector with a type 17 fiber enhances gene transfer to human airway epithelia.
Genetic Diseases, Inborn
A Homogeneous Cell-Based Halide-Sensitive Yellow Fluorescence Protein Assay to Identify Modulators of the Cystic Fibrosis Transmembrane Conductance Regulator Ion Channel.
Genetic Diseases, Inborn
A kinase-regulated mechanism controls CFTR channel gating by disrupting bivalent PDZ domain interactions.
Genetic Diseases, Inborn
A mutation of the epithelial sodium channel associated with atypical cystic fibrosis increases channel open probability and reduces Na+ self inhibition.
Genetic Diseases, Inborn
A Novel Cystic Fibrosis Gene Mutation C.4242+1G>C in an Omani Patient: A Case Report.
Genetic Diseases, Inborn
A novel regulatory role for tissue transglutaminase in epithelial-mesenchymal transition in cystic fibrosis.
Genetic Diseases, Inborn
A single amino acid substitution in CFTR converts ATP to an inhibitory ligand.
Genetic Diseases, Inborn
A SYK/SHC1 pathway regulates the amount of CFTR in the plasma membrane.
Genetic Diseases, Inborn
Adapting Proteostasis and Autophagy for Controlling the Pathogenesis of Cystic Fibrosis Lung Disease.
Genetic Diseases, Inborn
Alpha-1 Antitrypsin-A Target for MicroRNA-Based Therapeutic Development for Cystic Fibrosis.
Genetic Diseases, Inborn
Altering intracellular pH reveals the kinetic basis of intraburst gating in the CFTR Cl(-) channel.
Genetic Diseases, Inborn
Applicability of different antibodies for immunohistochemical localization of CFTR in sweat glands from healthy controls and from patients with cystic fibrosis.
Genetic Diseases, Inborn
Aquagenic Wrinkling of the Palm: A Rare Diagnostic Clue of Cystic Fibrosis and the Response to CFTR-Modulating Therapy.
Genetic Diseases, Inborn
Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis.
Genetic Diseases, Inborn
Azithromycin fails to reduce inflammation in cystic fibrosis airway epithelial cells.
Genetic Diseases, Inborn
Bone disease in cystic fibrosis: new pathogenic insights opening novel therapies.
Genetic Diseases, Inborn
Calreticulin negatively regulates the cell surface expression of cystic fibrosis transmembrane conductance regulator.
Genetic Diseases, Inborn
Capsaicin-Loaded Chitosan Nanocapsules for wtCFTR-mRNA Delivery to a Cystic Fibrosis Cell Line.
Genetic Diseases, Inborn
Cardiovascular complications in cystic fibrosis: A review of the literature.
Genetic Diseases, Inborn
Caring for Adolescents with Cystic Fibrosis, in Portugal: The Nurse's Role.
Genetic Diseases, Inborn
Cefoperazone sodium liposomal formulation to mitigate P. aeruginosa biofilm in Cystic fibrosis infection: A QbD approach.
Genetic Diseases, Inborn
Ceftolozane/tazobactam for pulmonary exacerbation in a 63-year-old cystic fibrosis patient with renal insufficiency and an elevated MIC to Pseudomonas aeruginosa.
Genetic Diseases, Inborn
CELF proteins regulate CFTR pre-mRNA splicing: essential role of the divergent domain of ETR-3.
Genetic Diseases, Inborn
Cell-Selective Regulation of CFTR Gene Expression: Relevance to Gene Editing Therapeutics.
Genetic Diseases, Inborn
CFTR dysfunction in cystic fibrosis and chronic obstructive pulmonary disease.
Genetic Diseases, Inborn
CFTR Modulators: Impact on Fertility, Pregnancy, and Lactation in Women with Cystic Fibrosis.
Genetic Diseases, Inborn
CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR.
Genetic Diseases, Inborn
Challenges Facing Airway Epithelial Cell-Based Therapy for Cystic Fibrosis.
Genetic Diseases, Inborn
Characterization of an intron 12 splice donor mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Genetic Diseases, Inborn
Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia.
Genetic Diseases, Inborn
Chloride Conductance, Nasal Potential Difference and Cystic Fibrosis Pathophysiology.
Genetic Diseases, Inborn
Cigarette Smoke and CFTR: Implications in the pathogenesis of COPD.
Genetic Diseases, Inborn
Clinically-approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia.
Genetic Diseases, Inborn
Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis.
Genetic Diseases, Inborn
Correction of the CF defect by curcumin: hypes and disappointments.
Genetic Diseases, Inborn
Corticosteroid use and increased CXCR2 levels on leukocytes are associated with lumacaftor/ivacaftor discontinuation in cystic fibrosis patients homozygous for the F508del CFTR mutation.
Genetic Diseases, Inborn
Current Status of Genetic Diagnosis Laboratories and Frequency of Genetic Variants Associated with Cystic Fibrosis through a Newborn-Screening Program in Turkey.
Genetic Diseases, Inborn
Cystic fibrosis and innate immunity: how chloride channel mutations provoke lung disease.
Genetic Diseases, Inborn
Cystic Fibrosis of the Pancreas: The Role of CFTR Channel in the Regulation of Intracellular Ca2+ Signaling and Mitochondrial Function in the Exocrine Pancreas.
Genetic Diseases, Inborn
Cystic fibrosis transmembrane conductance regulator degradation depends on the lectins Htm1p/EDEM and the Cdc48 protein complex in yeast.
Genetic Diseases, Inborn
Cystic Fibrosis Transmembrane Conductance Regulator Genotype, Not Circulating Catecholamines, Influences Cardiovascular Function in Patients with Cystic Fibrosis.
Genetic Diseases, Inborn
Cystic fibrosis transmembrane conductance regulator-emerging regulator of cancer.
Genetic Diseases, Inborn
Cystic Fibrosis: Overview of the Current Development Trends and Innovative Therapeutic Strategies.
Genetic Diseases, Inborn
Cystic fibrosis: Physiopathology and the latest pharmacological treatments.
Genetic Diseases, Inborn
Cytokine pattern in cystic fibrosis patients during antibiotic therapy and gene therapy using adenoviral vector.
Genetic Diseases, Inborn
Dataset of differential lipid raft and global proteomes of SILAC-labeled cystic fibrosis cells upon TNF -? stimulation.
Genetic Diseases, Inborn
Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition.
Genetic Diseases, Inborn
Defective CFTR-dependent CREB activation results in impaired spermatogenesis and azoospermia.
Genetic Diseases, Inborn
Deletion of cftr Leads to an Excessive Neutrophilic Response and Defective Tissue Repair in a Zebrafish Model of Sterile Inflammation.
Genetic Diseases, Inborn
Diagnostic tools and CFTR functional assays in cystic fibrosis: utility and availability in Switzerland.
Genetic Diseases, Inborn
Differential function of the two nucleotide binding domains on cystic fibrosis transmembrane conductance regulator.
Genetic Diseases, Inborn
Differential sensitivity of the cystic fibrosis (CF)-associated mutants G551D and G1349D to potentiators of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel.
Genetic Diseases, Inborn
Discovery of GLPG2451, a Novel Once Daily Potentiator for the Treatment of Cystic Fibrosis.
Genetic Diseases, Inborn
Dysfunction of the non-neuronal cholinergic system in the airways and blood cells of patients with cystic fibrosis.
Genetic Diseases, Inborn
Dysregulated signalling pathways in innate immune cells with cystic fibrosis mutations.
Genetic Diseases, Inborn
Electrophysiological Approaches for the Study of Ion Channel Function.
Genetic Diseases, Inborn
Elevated tear fluid levels of MIP-1alpha in patients with cystic fibrosis.
Genetic Diseases, Inborn
Epithelial mesenchymal transition (EMT): a universal process in lung diseases with implications for cystic fibrosis pathophysiology.
Genetic Diseases, Inborn
Establishment and characterization of a novel polarized MDCK epithelial cellular model for CFTR studies.
Genetic Diseases, Inborn
Evolving Mechanistic Views and Emerging Therapeutic Strategies for Cystic Fibrosis-Related Diabetes.
Genetic Diseases, Inborn
Fas and Fas ligand expression in cystic fibrosis airway epithelium.
Genetic Diseases, Inborn
Fas Expression in Conjunctival Epithelial Cells of Patients With Cystic Fibrosis.
Genetic Diseases, Inborn
Folding and rescue of a cystic fibrosis transmembrane conductance regulator trafficking mutant identified using human-murine chimeric proteins.
Genetic Diseases, Inborn
Functional characterization reveals that zebrafish CFTR prefers to occupy closed channel conformations.
Genetic Diseases, Inborn
Functional Gene Correction for Cystic Fibrosis in Lung Epithelial Cells Generated from Patient iPSCs.
Genetic Diseases, Inborn
Generation of an induced pluripotent stem cell line (MHHi018-A) from a patient with Cystic Fibrosis carrying p.Asn1303Lys (N1303K) mutation.
Genetic Diseases, Inborn
Glibenclamide stimulates fluid secretion in rodent cholangiocytes through a cystic fibrosis transmembrane conductance regulator-independent mechanism.
Genetic Diseases, Inborn
High-Throughput Screening for Readthrough Modulators of CFTR PTC Mutations.
Genetic Diseases, Inborn
Human Genetics Society of Australasia position statement: population-based carrier screening for cystic fibrosis.
Genetic Diseases, Inborn
Identification and membrane localization of electrogenic sodium bicarbonate cotransporters in Calu-3 cells.
Genetic Diseases, Inborn
Immune response of polarized cystic fibrosis airway epithelial cells infected with Influenza A virus.
Genetic Diseases, Inborn
Impact of the F508del mutation on ovine CFTR, a Cl(-) channel with enhanced conductance and ATP-dependent gating.
Genetic Diseases, Inborn
Impaired CFTR-Dependent Amplification of FSH-Stimulated Estrogen Production in Cystic Fibrosis and PCOS.
Genetic Diseases, Inborn
In vivo evaluation of the safety of adenovirus-mediated transfer of the human cystic fibrosis transmembrane conductance regulator cDNA to the lung.
Genetic Diseases, Inborn
In-vivo crystals reveal critical features of the interaction between CFTR and the PDZ2 domain of Na+/H+ exchange cofactor NHERF1.
Genetic Diseases, Inborn
Incidence, Prevalence, Etiology, and Prognosis of First-Time Chronic Pancreatitis in Young Patients: A Nationwide Cohort Study.
Genetic Diseases, Inborn
Increased arylsulfatase B activity in cystic fibrosis cells following correction of CFTR.
Genetic Diseases, Inborn
Increased Folding and Channel Activity of a Rare Cystic Fibrosis Mutant with CFTR Modulators.
Genetic Diseases, Inborn
Insight into cystic fibrosis by structural modelling of CFTR first nucleotide binding fold (NBF1).
Genetic Diseases, Inborn
Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis.
Genetic Diseases, Inborn
Iron chelation as novel treatment for lung inflammation in cystic fibrosis.
Genetic Diseases, Inborn
Mechanosensitivity of wild-type and G551D cystic fibrosis transmembrane conductance regulator (CFTR) controls regulatory volume decrease in simple epithelia.
Genetic Diseases, Inborn
Metabolic Reprograming of Cystic Fibrosis Macrophages via the IRE1? Arm of the Unfolded Protein Response Results in Exacerbated Inflammation.
Genetic Diseases, Inborn
Mitochondrial oxidative stress in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice.
Genetic Diseases, Inborn
Mitochondrial Stress Responses and "Mito-Inflammation" in Cystic Fibrosis.
Genetic Diseases, Inborn
Modeling the Conformational Changes Underlying Channel Opening in CFTR.
Genetic Diseases, Inborn
Molecular Dynamics Flexible Fitting Simulations Identify New Models of the Closed State of the Cystic Fibrosis Transmembrane Conductance Regulator Protein.
Genetic Diseases, Inborn
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin.
Genetic Diseases, Inborn
Nanoparticles that deliver triplex-forming peptide nucleic acid molecules correct F508del CFTR in airway epithelium.
Genetic Diseases, Inborn
Non-specific activation of the epithelial sodium channel by the CFTR chloride channel.
Genetic Diseases, Inborn
Novel frameshift variant of the CFTR gene: S511Lfs*2 from phenotype to molecular predictions.
Genetic Diseases, Inborn
Novel Hits in the Correction of ?F508-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Protein: Synthesis, Pharmacological, and ADME Evaluation of Tetrahydropyrido[4,3-d]pyrimidines for the Potential Treatment of Cystic Fibrosis.
Genetic Diseases, Inborn
Nutritional effects on host response to lung infections with mucoid Pseudomonas aeruginosa in mice.
Genetic Diseases, Inborn
On the discovery and development of CFTR chloride channel activators.
Genetic Diseases, Inborn
On the interactions between nucleotide binding domains and membrane spanning domains in cystic fibrosis transmembrane regulator: A molecular dynamic study.
Genetic Diseases, Inborn
PDE5 inhibitors for cystic fibrosis: can they also enhance chloride transport? Evaluation of: Lubamba B, Lecourt H, Lebacq J, et al. Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis. Am J Respir Crit Care Med 2008;177(5):506-15.
Genetic Diseases, Inborn
Pharmacological analysis of CFTR variants of cystic fibrosis using stem cell-derived organoids.
Genetic Diseases, Inborn
Pharmacological approaches to correcting the ion transport defect in cystic fibrosis.
Genetic Diseases, Inborn
Phenotypic and Genetic Characterization of Carbapenemase and ESBLs Producing Gram-negative Bacteria (GNB) Isolated from Patients with Cystic Fibrosis (CF) in Tehran Hospitals.
Genetic Diseases, Inborn
Pitfalls in the immunohistochemical localization of the cystic fibrosis transmembrane conductance regulator in paraffin embedded sweat glands.
Genetic Diseases, Inborn
Potentiation of the cystic fibrosis transmembrane conductance regulator by VX-770 involves stabilization of the pre-hydrolytic, O1 state.
Genetic Diseases, Inborn
Pseudomonas aeruginosa triggers CFTR-mediated airway surface liquid secretion in swine trachea.
Genetic Diseases, Inborn
Pulmonary bacterial pathogens in cystic fibrosis patients and antibiotic therapy: a tool for the health workers.
Genetic Diseases, Inborn
RACK1 interacts with filamin-A to regulate plasma membrane levels of the cystic fibrosis transmembrane conductance regulator.
Genetic Diseases, Inborn
Recent Progress in the Discovery and Development of Small-Molecule Modulators of CFTR.
Genetic Diseases, Inborn
Relating the Disease Mutation Spectrum to the Evolution of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Genetic Diseases, Inborn
Resveratrol rescues cAMP-dependent anionic transport in the cystic fibrosis pancreatic cell line CFPAC1.
Genetic Diseases, Inborn
Role of individual R domain phosphorylation sites in CFTR regulation by protein kinase A.
Genetic Diseases, Inborn
Role of the amiloride-sensitive epithelial Na+ channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease.
Genetic Diseases, Inborn
Selectively targeting key inflammatory pathways in cystic fibrosis.
Genetic Diseases, Inborn
Short communication: novel truncating mutations in the CFTR gene causing a severe form of cystic fibrosis in Italian patients.
Genetic Diseases, Inborn
Sleep-Related Rhythmic Movement Disorder in Triplets: Evidence for Genetic Predisposition?
Genetic Diseases, Inborn
Small Molecule Anion Carriers Correct Abnormal Airway Surface Liquid Properties in Cystic Fibrosis Airway Epithelia.
Genetic Diseases, Inborn
Specific resistance to Pseudomonas aeruginosa infection in zebrafish is mediated by the cystic fibrosis transmembrane conductance regulator.
Genetic Diseases, Inborn
Structural models of CFTR-AMPK and CFTR-PKA interactions: R-domain flexibility is a key factor in CFTR regulation.
Genetic Diseases, Inborn
Subepithelial fibrosis and degradation of the bronchial extracellular matrix in cystic fibrosis.
Genetic Diseases, Inborn
Synthesis and biological evaluation of thiazole derivatives on basic defects underlying cystic fibrosis.
Genetic Diseases, Inborn
Synthesis and properties of molecular probes for the rescue site on mutant cystic fibrosis transmembrane conductance regulator.
Genetic Diseases, Inborn
Testicular Cancer in a Lung Transplant Patient With Cystic Fibrosis: A Case Report.
Genetic Diseases, Inborn
The Application of Bicarbonate Recovers the Chemical-Physical Properties of Airway Surface Liquid in Cystic Fibrosis Epithelia Models.
Genetic Diseases, Inborn
The cystic fibrosis mutation G1349D within the signature motif LSHGH of NBD2 abolishes the activation of CFTR chloride channels by genistein.
Genetic Diseases, Inborn
The Role of Specialized Pro-Resolving Mediators in Cystic Fibrosis Airways Disease.
Genetic Diseases, Inborn
Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion.
Genetic Diseases, Inborn
Transmembrane domain of cystic fibrosis transmembrane conductance regulator: design, characterization, and secondary structure of synthetic peptides m1-m6.
Genetic Diseases, Inborn
Triplet CFTR modulators: future prospects for treatment of cystic fibrosis.
Genetic Diseases, Inborn
Unplugging Mucus in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Genetic Diseases, Inborn
Unravelling the Regions of Mutant F508del-CFTR More Susceptible to the Action of Four Cystic Fibrosis Correctors.
Genetic Diseases, Inborn
Unveiling the degradative route of the V247M ?-sarcoglycan mutant responsible for LGMD-2D.
Genetic Diseases, Inborn
Uptake of fluorescent dyes associated with the functional expression of the cystic fibrosis transmembrane conductance regulator in epithelial cells.
Genetic Diseases, Inborn
Use of a High-Throughput Phenotypic Screening Strategy to Identify Amplifiers, a Novel Pharmacological Class of Small Molecules That Exhibit Functional Synergy with Potentiators and Correctors.
Genetic Diseases, Inborn
Widespread airway distribution and short-term phenotypic correction of cystic fibrosis pigs following aerosol delivery of piggyBac/adenovirus.
Genetic Diseases, Inborn
Widespread and efficient marker gene expression in the airway epithelia of fetal sheep after minimally invasive tracheal application of recombinant adenovirus in utero.
Genetic Diseases, Inborn
[Antisense oligonucleotides for therapy of cystic fibrosis : Inhibition of sodium absorption mediated by ENaC in nasal epithelial cells.]
Genetic Diseases, Inborn
[Microbiological diagnosis of bronchopulmonary colonization-infection in cystic fibrosis.]
Genetic Diseases, Inborn
[Post-translational ligation and function of dual-vector transferred split CFTR gene].
Genetic Diseases, Inborn
[Post-translational ligation of split CFTR severed before TMD2 and its chloride channel function].
Gingivitis
ANALYSIS OF LOCAL IMMUNITY INDICATORS OF THE ORAL CAVITY AND DEGREE OF GINGIVITIS DEPENDING ON MUTATION OF CFTR GENE IN CHILDREN WITH CYSTIC FIBROSIS.
Glioblastoma
CFTR activation suppresses glioblastoma cell proliferation, migration and invasion.
Glioma
CFTR promotes malignant glioma development via up-regulation of Akt/Bcl2-mediated anti-apoptosis pathway.
Glioma
Chlorotoxin does not inhibit volume-regulated, calcium-activated and cyclic AMP-activated chloride channels.
Glycogen Storage Disease Type VI
Cholestasis in Benign Recurrent Intrahepatic Cholestasis 2.
Heart Failure
Loss of the normal epicardial to endocardial gradient of cftr mRNA expression in the hypertrophied rabbit left ventricle.
Heart Failure
Tumor necrosis factor-?-mediated downregulation of the cystic fibrosis transmembrane conductance regulator drives pathological sphingosine-1-phosphate signaling in a mouse model of heart failure.
Hemochromatosis
Fluorescence energy transfer detection as a homogeneous DNA diagnostic method.
Hepatoblastoma
Glibenclamide induces apoptosis through inhibition of cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channels and intracellular Ca(2+) release in HepG2 human hepatoblastoma cells.
Hepatoblastoma
Role of pertussis toxin-sensitive G-proteins in intracellular Ca2+ release and apoptosis induced by inhibiting cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channels in HepG2 human hepatoblastoma cells.
Herpes Zoster
Effects and interaction of dietary electrolyte balance and citric acid on the intestinal function of weaned piglets.
Herpes Zoster
In vivo investigation of acidified pepsin exposure to porcine vocal fold epithelia.
Herpes Zoster
Mechanisms of attenuation of abdominal sepsis induced acute lung injury by ascorbic acid.
Hyperhomocysteinemia
Folate Protects Hepatocytes of Hyperhomocysteinemia Mice From Apoptosis via Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Activated Endoplasmic Reticulum Stress.
Hypertension, Portal
Obliterative Portal Venopathy Without Cirrhosis Is Prevalent in Pediatric Cystic Fibrosis Liver Disease With Portal Hypertension.
Hypokalemia
A specific cystic fibrosis mutation (T3381) associated with the phenotype of isolated hypotonic dehydration.
Hyponatremia
A specific cystic fibrosis mutation (T3381) associated with the phenotype of isolated hypotonic dehydration.
Hypothyroidism
Altered ion transport by thyroid epithelia fromCFTR-/- pigs suggests mechanisms for hypothyroidism in Cystic Fibrosis.
Infections
16alpha-Bromoepiandrosterone (HE2000) limits non-productive inflammation and stimulates immunity in lungs.
Infections
A Link Between a Common Mutation in CFTR and Impaired Innate and Adaptive Viral Defense.
Infections
AAV exploits subcellular stress associated with inflammation, endoplasmic reticulum expansion, and misfolded proteins in models of cystic fibrosis.
Infections
Abnormally up-regulated cystic fibrosis transmembrane conductance regulator expression and uterine fluid accumulation contribute to Chlamydia trachomatis-induced female infertility.
Infections
Absence of the cystic fibrosis transmembrane regulator (Cftr) from myeloid-derived cells slows resolution of inflammation and infection.
Infections
Accumulation of ceramide in the trachea and intestine of cystic fibrosis mice causes inflammation and cell death.
Infections
Acute pancreatitis in association with Campylobacter jejuni-associated diarrhea in a 15-year-old with CFTR mutations: is there a link?
Infections
Aerosolized Bovine Lactoferrin Counteracts Infection, Inflammation and Iron Dysbalance in A Cystic Fibrosis Mouse Model of Pseudomonas aeruginosa Chronic Lung Infection.
Infections
Airway epithelial control of Pseudomonas aeruginosa infection in cystic fibrosis.
Infections
Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies.
Infections
Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis.
Infections
Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator.
Infections
Burkholderia cenocepacia O polysaccharide chain contributes to caspase-1-dependent IL-1beta production in macrophages.
Infections
Cefoperazone sodium liposomal formulation to mitigate P. aeruginosa biofilm in Cystic fibrosis infection: A QbD approach.
Infections
Ceftolozane/tazobactam for pulmonary exacerbation in a 63-year-old cystic fibrosis patient with renal insufficiency and an elevated MIC to Pseudomonas aeruginosa.
Infections
Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis.
Infections
CFTR mutations and host susceptibility to Pseudomonas aeruginosa lung infection.
Infections
Characterization of CFTR expression in a human pulmonary mucoepidermoid carcinoma cell line, NCI-H292 cells.
Infections
Clusters of Cl- channels in CFTR-expressing Sf9 cells switch spontaneously between slow and fast gating modes.
Infections
Common CFTR gene variants influence body composition and survival in rural Ghana.
Infections
Concurrent amyotrophic lateral sclerosis and cystic fibrosis supports common pathways of pathogenesis.
Infections
Cystic fibrosis transmembrane conductance regulator (CFTR)-mediated residual chloride secretion does not protect against early chronic Pseudomonas aeruginosa infection in F508del homozygous cystic fibrosis patients.
Infections
Cystic fibrosis transmembrane conductance regulator is an epithelial cell receptor for clearance of Pseudomonas aeruginosa from the lung.
Infections
Cystic Fibrosis Transmembrane Conductance Regulator Reduces Microtubule-Dependent Campylobacter jejuni Invasion.
Infections
Cystic fibrosis year in review 2019: Section 3 multisystem-based care and research.
Infections
Disruption of CFTR-dependent lipid rafts reduces bacterial levels and corneal disease in a murine model of Pseudomonas aeruginosa keratitis.
Infections
Effects of rhinovirus infection on the expression and function of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel in human nasal mucosa.
Infections
Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa.
Infections
Gene therapy in a xenograft model of cystic fibrosis lung corrects chloride transport more effectively than the sodium defect.
Infections
Genomewide association analysis of respiratory syncytial virus infection in mice.
Infections
Helicobacter pylori infection downregulates duodenal CFTR and SLC26A6 expressions through TGF? signaling pathway.
Infections
How mutant CFTR may contribute to Pseudomonas aeruginosa infection in cystic fibrosis.
Infections
Immune response of polarized cystic fibrosis airway epithelial cells infected with Influenza A virus.
Infections
In vitro assessment of variables affecting the efficiency and efficacy of adenovirus-mediated gene transfer to cystic fibrosis airway epithelia.
Infections
Infection of Polarized Airway Epithelial Cells by Normal and Small-Colony Variant Strains of Staphylococcus aureus Is Increased in Cells with Abnormal Cystic Fibrosis Transmembrane Conductance Regulator Function and Is Influenced by NF-{kappa}B.
Infections
Inflammatory epithelial cytokines after in vitro respiratory syncytial viral infection are associated with reduced lung function.
Infections
Influence of cystic fibrosis transmembrane conductance regulator on gene expression in response to Pseudomonas aeruginosa infection of human bronchial epithelial cells.
Infections
Influenza virus M2 targets cystic fibrosis transmembrane conductance regulator for lysosomal degradation during viral infection.
Infections
Innovating cystic fibrosis clinical trial designs in an era of successful standard of care therapies.
Infections
Involvement of cystic fibrosis transmembrane conductance regulator (CFTR) in the pathogenesis of hydrosalpinx induced by Chlamydia trachomatis infection.
Infections
Involvement of cystic fibrosis transmembrane conductance regulator in infection-induced edema.
Infections
Mid-trimester hyperechogenic bowel in a fetus of Japanese origin carrying a new mutation of CFTR gene (L548Q).
Infections
Mucociliary clearance in cystic fibrosis knockout mice infected with Pseudomonas aeruginosa.
Infections
Nutritional effects on host response to lung infections with mucoid Pseudomonas aeruginosa in mice.
Infections
Optimal Complement-Mediated Phagocytosis of Pseudomonas aeruginosa by Monocytes Is Cystic Fibrosis Transmembrane Conductance Regulator-Dependent.
Infections
Pathogen acquisition in patients with cystic fibrosis receiving ivacaftor or lumacaftor/ivacaftor.
Infections
Physiological impact of abnormal lipoxin A? production on cystic fibrosis airway epithelium and therapeutic potential.
Infections
Progression of early structural lung disease in young children with cystic fibrosis assessed using CT.
Infections
Prospective analysis of cystic fibrosis transmembrane regulator mutations in adults with bronchiectasis or pulmonary nontuberculous mycobacterial infection.
Infections
Pseudomonas aeruginosa alginate promotes Burkholderia cenocepacia persistence in cystic fibrosis transmembrane conductance regulator knockout mice.
Infections
Pulmonary nontuberculous mycobacterial disease: prospective study of a distinct preexisting syndrome.
Infections
Reduced GM1 Ganglioside in CFTR-Deficient Human Airway Cells Results in Decreased ss1-integrin Signaling and Delayed Wound Repair.
Infections
Regulation of Pseudomonas aeruginosa internalization after contact lens wear in vivo and in serum-free culture by ocular surface cells.
Infections
Regulation of the Coxsackie and adenovirus receptor expression is dependent on cystic fibrosis transmembrane regulator in airway epithelial cells.
Infections
Respiratory syncytial virus infection disrupts monolayer integrity and function in cystic fibrosis airway cells.
Infections
Response to Acute Lung Infection with Mucoid Pseudomonas aeruginosa in Cystic Fibrosis Mice.
Infections
Restoration of cyclic adenosine monophosphate-stimulated chloride channel activity in human cystic fibrosis tracheobronchial submucosal gland cells by adenovirus-mediated and cationic lipid-mediated gene transfer.
Infections
Rhesus ?-defensin-1 (RTD-1) exhibits in vitro and in vivo activity against cystic fibrosis strains of Pseudomonas aeruginosa.
Infections
Role for cystic fibrosis transmembrane conductance regulator protein in a glutathione response to bronchopulmonary pseudomonas infection.
Infections
Role of CFTR expressed by neutrophils in modulating acute lung inflammation and injury in mice.
Infections
Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections.
Infections
Role of the cystic fibrosis transmembrane conductance regulator in internalization of Pseudomonas aeruginosa by polarized respiratory epithelial cells.
Infections
Specific resistance to Pseudomonas aeruginosa infection in zebrafish is mediated by the cystic fibrosis transmembrane conductance regulator.
Infections
Src signaling links mediators of inflammation to Cx43 gap junction channels in primary and transformed CFTR-expressing airway cells.
Infections
The Sweat Metabolome of Screen-Positive Cystic Fibrosis Infants: Revealing Mechanisms beyond Impaired Chloride Transport.
Infections
Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.
Infections
Ursodeoxycholic acid inhibits ENaC and Na/K pump activity to restore airway surface liquid height in cystic fibrosis bronchial epithelial cells.
Infections
Variants in the glutamate-cysteine-ligase gene are associated with cystic fibrosis lung disease.
Infertility
Analysis of the whole CFTR coding regions and splice junctions in azoospermic men with congenital bilateral aplasia of epididymis or vas deferens.
Infertility
Clinical genetic testing for male factor infertility: current applications and future directions.
Infertility
Cystic fibrosis transmembrane conductance regulator mutations in azoospermic and oligospermic men and their partners.
Infertility
Differential cellular expression of cystic fibrosis transmembrane regulator in human reproductive tissues. Clues for the infertility in patients with cystic fibrosis.
Infertility
Early activation of sperm by HCO(3)(-) is regulated hormonally in the murine uterus.
Infertility
Endocervical metaplasia of the endometrium in a patient with cystic fibrosis: a case report.
Infertility
Female cystic fibrosis mutation carriers and assisted reproductive technology: does carrier status affect reproductive outcomes?
Infertility
Gender-sensitive association of CFTR gene mutations and 5T allele emerging from a large survey on infertility.
Infertility
Involvement of CFTR in oviductal HCO3- secretion and its effect on soluble adenylate cyclase-dependent early embryo development.
Infertility
Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report.
Infertility
Loss of SLC9A3 decrease CFTR protein and causes obstructed azoospermia in mice.
Infertility
Screening for mutations in the cystic fibrosis transmembrane regulator gene in an infertility clinic.
Infertility
The decline of fertility in male uremic patients is correlated with low expression of the cystic fibrosis transmembrane conductance regulator protein (CFTR) in human sperm.
Infertility, Female
Abnormally up-regulated cystic fibrosis transmembrane conductance regulator expression and uterine fluid accumulation contribute to Chlamydia trachomatis-induced female infertility.
Infertility, Male
A mutation in the cystic fibrosis transmembrane conductance regulator gene associated with elevated sweat chloride concentrations in the absence of cystic fibrosis.
Infertility, Male
A novel mutation (-195C>A) in the promoter region of CFTR gene is associated with Chinese Congenital Bilateral Absence of Vas Deferens (CBAVD).
Infertility, Male
Carbamazepine-induced sperm disorders can be associated with the altered expressions of testicular KCNJ11/miR-let-7a and spermatozoal CFTR/miR-27a.
Infertility, Male
Clinical genetic testing for male factor infertility: current applications and future directions.
Infertility, Male
Cystic fibrosis gene mutations and polymorphisms in Saudi men with infertility.
Infertility, Male
Genetic cystic fibrosis transmembrane regulator 4016insT D1152H compound heterozygosity and male infertility: an Italian case report.
Infertility, Male
Genetic markers of male infertility: Y chromosome microdeletions and cystic fibrosis transmembrane conductance gene mutations.
Infertility, Male
Impact of Cystic Fibrosis Transmembrane Regulator (CFTR) gene mutations on male infertility.
Infertility, Male
Is intracytoplasmic sperm injection itself an indication to perform preimplantation genetic diagnosis (PGD)? About PGD, invasive prenatal diagnosis and genetic sonography.
Infertility, Male
Linkage disequilibrium between the M470V variant and the IVS8 polyT alleles of the CFTR gene in CBAVD.
Infertility, Male
Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis.
Infertility, Male
Molecular analysis of mutations and polymorphisms in the CFTR gene in male infertility.
Infertility, Male
Molecular analysis of the IVS8-T splice variant 5T and M470V exon 10 missense polymorphism in Iranian males with congenital bilateral absence of the vas deferens.
Infertility, Male
Molecular screening of the CFTR gene in men with anomalies of the vas deferens: identification of three novel mutations.
Infertility, Male
Mutation Studies in the CFTR Gene in Asian Indian Subjects with Congenital Bilateral Absence of Vas Deferens: Report of Two Novel Mutations and Four Novel Variants.
Infertility, Male
Mutations in CFTR gene and clinical correlation in Argentine patients with congenital bilateral absence of the vas deferens.
Infertility, Male
Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens.
Infertility, Male
Regulation of male fertility by CFTR and implications in male infertility.
Infertility, Male
Screening of ?F508 mutation and IVS8-poly T polymorphism in CFTR gene in Tunisian infertile men without CBAVD.
Infertility, Male
Study of mutant and polyvariant mutant CFTR genes in patients with congenital absence of the vas deferens.
Infertility, Male
The CFTR Met 470 allele is associated with lower birth rates in fertile men from a population isolate.
Inflammatory Bowel Diseases
Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis.
Influenza, Human
Delivery of purified, functional CFTR to epithelial cells in vitro using influenza hemagglutinin.
Influenza, Human
Heterozygosity for the F508del mutation in the cystic fibrosis transmembrane conductance regulator anion channel attenuates influenza severity.
Influenza, Human
Influenza virus M2 targets cystic fibrosis transmembrane conductance regulator for lysosomal degradation during viral infection.
Influenza, Human
TGF-?-induced IL-6 prevents development of acute lung injury in influenza A virus-infected F508del CFTR-heterozygous mice.
Insulin Resistance
The ?F508 Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator Is Associated With Progressive Insulin Resistance and Decreased Functional ?-Cell Mass in Mice.
Intestinal Diseases
Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota.
Intestinal Diseases
Toll-like receptor-4 genotype influences the survival of cystic fibrosis mice.
Intestinal Obstruction
Accumulation of ceramide in the trachea and intestine of cystic fibrosis mice causes inflammation and cell death.
Intestinal Obstruction
Clinical and genetic risk factors for cystic fibrosis-related liver disease.
Intestinal Obstruction
Distal intestinal obstruction syndrome in adults with cystic fibrosis.
Intestinal Obstruction
Growth deficits in cystic fibrosis mice begin in utero prior to IGF-1 reduction.
Intestinal Obstruction
Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor.
Irritable Bowel Syndrome
Inhibition of Na+ /H+ exchanger isoform 3 improves gut fluidity and alkalinity in cystic fibrosis transmembrane conductance regulator-deficient and F508del mutant mice.
Klinefelter Syndrome
Transmission of male infertility and intracytoplasmic sperm injection (mini-review).
Leukemia
High-expressing cystic fibrosis transmembrane conductance regulator interacts with histone deacetylase 2 to promote the development of Ph+ leukemia through the HDAC2-mediated PTEN pathway.
Leukemia
[Relationship of Cystic Fibrosis Transmembrane Conductance Regulator Expression with Clinical Features and Prognosis in Patients with Acute Leukemia].
Liver Diseases
CFTR expression and mucin secretion in cultured mouse gallbladder epithelial cells.
Liver Diseases
Elevated hepatic SULT1E1 activity in mouse models of cystic fibrosis alters the regulation of estrogen responsive proteins.
Liver Diseases
Glibenclamide stimulates fluid secretion in rodent cholangiocytes through a cystic fibrosis transmembrane conductance regulator-independent mechanism.
Liver Diseases
Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report.
Liver Diseases
Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR.
Liver Diseases
Multi-dimensional clinical phenotyping of a national cohort of adult cystic fibrosis patients.
Liver Diseases
Obliterative Portal Venopathy Without Cirrhosis Is Prevalent in Pediatric Cystic Fibrosis Liver Disease With Portal Hypertension.
Liver Diseases
Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases.
Liver Diseases
Unusual Cystic Fibrosis Transmembrane Conductance Regulator Mutations and Liver Disease: A Case Series and Review of the Literature.
Lung Diseases
A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease.
Lung Diseases
A placebo-controlled study of liposome-mediated gene transfer to the nasal epithelium of patients with cystic fibrosis.
Lung Diseases
Aspergillus-induced superoxide production by cystic fibrosis phagocytes is associated with disease severity.
Lung Diseases
Association of cystic fibrosis genetic modifiers with congenital bilateral absence of the vas deferens.
Lung Diseases
Associations between peak oxygen uptake, lung function, and bronchiectasis in children with cystic fibrosis in the era of CFTR modulators.
Lung Diseases
Autophagy Augmentation to Alleviate Immune Response Dysfunction, and Resolve Respiratory and COVID-19 Exacerbations.
Lung Diseases
CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells.
Lung Diseases
CFTR gene mutations--including three novel nucleotide substitutions--and haplotype background in patients with asthma, disseminated bronchiectasis and chronic obstructive pulmonary disease.
Lung Diseases
CFTR transgene expression in primary DeltaF508 epithelial cell cultures from human nasal polyps following gene transfer with cationic phosphonolipids.
Lung Diseases
CFTR-deficient pigs display alterations of bone microarchitecture and composition at birth.
Lung Diseases
CFTR-function and ventilation inhomogeneity in individuals with cystic fibrosis.
Lung Diseases
Cigarette Smoke-Induced Acquired Dysfunction of Cystic Fibrosis Transmembrane Conductance Regulator in the Pathogenesis of Chronic Obstructive Pulmonary Disease.
Lung Diseases
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
Lung Diseases
Cystic fibrosis swine fail to secrete airway surface liquid in response to inhalation of pathogens.
Lung Diseases
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Asians with chronic pulmonary disease: a pilot study.
Lung Diseases
Cystic Fibrosis Transmembrane Conductance Regulator-independent Phagosomal Acidification in Macrophages.
Lung Diseases
Cystic Fibrosis Transmembrane Conductance Regulator. Implications in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Lung Diseases
Cystic fibrosis with three mutations in the cystic fibrosis transmembrane conductance regulator gene.
Lung Diseases
Cystic fibrosis year in review 2019: Section 3 multisystem-based care and research.
Lung Diseases
Cystic fibrosis: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases.
Lung Diseases
DEFECTIVE ORGANELLAR ACIDIFICATION AS A CAUSE OF CYSTIC FIBROSIS LUNG DISEASE - RE-EXAMINATION OF A RECURRING HYPOTHESIS.
Lung Diseases
Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function.
Lung Diseases
Designing Clinical Trials for Anti-Inflammatory Therapies in Cystic Fibrosis.
Lung Diseases
Detection of modifier loci influencing the lung phenotype of cystic fibrosis knockout mice.
Lung Diseases
Discovery of Icenticaftor (QBW251), a Cystic Fibrosis Transmembrane Conductance Regulator Potentiator with Clinical Efficacy in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Lung Diseases
Discovery of Multitarget Agents Active as Broad-Spectrum Antivirals and Correctors of Cystic Fibrosis Transmembrane Conductance Regulator for Associated Pulmonary Diseases.
Lung Diseases
EDNRA variants associate with smooth muscle mRNA levels, cell proliferation rates, and cystic fibrosis pulmonary disease severity.
Lung Diseases
Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host defense.
Lung Diseases
Evidence for safety and efficacy of DOTAP cationic liposome mediated CFTR gene transfer to the nasal epithelium of patients with cystic fibrosis.
Lung Diseases
Expression of alpha v beta 5 integrin is necessary for efficient adenovirus-mediated gene transfer in the human airway.
Lung Diseases
Expression of cystic fibrosis transmembrane conductance regulator in the human distal lung.
Lung Diseases
Expression of S100A8 correlates with inflammatory lung disease in congenic mice deficient of the cystic fibrosis transmembrane conductance regulator.
Lung Diseases
Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis.
Lung Diseases
Genotype-phenotype relationships in a cohort of adult cystic fibrosis patients.
Lung Diseases
Harnessing Neutrophil Survival Mechanisms during Chronic Infection by Pseudomonas aeruginosa: Novel Therapeutic Targets to Dampen Inflammation in Cystic Fibrosis.
Lung Diseases
How do cystic fibrosis transmembrane conductance regulator mutations produce lung disease?
Lung Diseases
Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance.
Lung Diseases
Hypertonic saline increases lung epithelial lining fluid glutathione and thiocyanate: two protective CFTR-dependent thiols against oxidative injury.
Lung Diseases
In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis.
Lung Diseases
Increasing the Endoplasmic Reticulum Pool of the F508del Allele of the Cystic Fibrosis Transmembrane Conductance Regulator Leads to Greater Folding Correction by Small Molecule Therapeutics.
Lung Diseases
Inefficient gene transfer by adenovirus vector to cystic fibrosis airway epithelia of mice and humans.
Lung Diseases
Inhaled interventions in cystic fibrosis: mucoactive and antibiotic therapies.
Lung Diseases
Inhibition of histone-deacetylase activity rescues inflammatory cystic fibrosis lung disease by modulating innate and adaptive immune responses.
Lung Diseases
Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis.
Lung Diseases
Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report.
Lung Diseases
Loss of Cftr function exacerbates the phenotype of Na+ hyperabsorption in murine airways.
Lung Diseases
Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis.
Lung Diseases
Nicotine Induces Progressive Properties of Lung Adenocarcinoma A549 Cells by Inhibiting Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Expression and Plasma Membrane Localization.
Lung Diseases
Nitric oxide synthase 1 as a potential modifier gene of decline in lung function in patients with cystic fibrosis.
Lung Diseases
Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia.
Lung Diseases
Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTATM.
Lung Diseases
Proteases and antiproteases in cystic fibrosis: pathogenetic considerations and therapeutic strategies.
Lung Diseases
Quantification of Phenotypic Variability of Lung Disease in Children with Cystic Fibrosis.
Lung Diseases
Relations between the frequency of the DeltaF 508 mutation and the course of pulmonary disease in cystic fibrosis patients infected with Pseudomonas aeruginosa.
Lung Diseases
Respiratory epithelial gene expression in patients with mild and severe cystic fibrosis lung disease.
Lung Diseases
Role of Binding and Nucleoside Diphosphate Kinase A in the Regulation of the Cystic Fibrosis Transmembrane Conductance Regulator by AMP-Activated Protein Kinase.
Lung Diseases
Safety of aerosolized INS 365 in patients with mild to moderate cystic fibrosis: results of a phase I multi-center study.
Lung Diseases
Sphingolipids and plasma membrane hydrolases in human primary bronchial cells during differentiation and their altered patterns in cystic fibrosis.
Lung Diseases
SPX-101 Is a Novel Epithelial Sodium Channel-targeted Therapeutic for Cystic Fibrosis That Restores Mucus Transport.
Lung Diseases
Strain-dependent pulmonary gene expression profiles of a cystic fibrosis mouse model.
Lung Diseases
The CFTR Mutation c.3453G > C (D1152H) Confers an Anion Selectivity Defect in Primary Airway Tissue that Can Be Rescued by Ivacaftor.
Lung Diseases
The effect of chloride concentration on human neutrophil functions: potential relevance to cystic fibrosis.
Lung Diseases
The need for vigilance: the case of a false-negative newborn screen for cystic fibrosis.
Lung Diseases
The yin and yang of cystic fibrosis transmembrane conductance regulator function: implications for chronic lung disease.
Lung Diseases
Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.
Lung Diseases
Unplugging Mucus in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Lung Diseases
Variants in the glutamate-cysteine-ligase gene are associated with cystic fibrosis lung disease.
Lung Diseases
Vector-specific complementation profiles of two independent primary defects in cystic fibrosis airways.
Lung Diseases, Obstructive
Cystic Fibrosis Transmembrane Conductance Regulator. Implications in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Lung Diseases, Obstructive
Increased incidence of cystic fibrosis gene mutations in adults with disseminated bronchiectasis.
Lung Injury
Association of cystic fibrosis transmembrane conductance regulator gene variants with acute lung injury in African American children with pneumonia.
Lung Injury
Critical modifier role of membrane-cystic fibrosis transmembrane conductance regulator-dependent ceramide signaling in lung injury and emphysema.
Lung Injury
Ursodeoxycholic acid inhibits ENaC and Na/K pump activity to restore airway surface liquid height in cystic fibrosis bronchial epithelial cells.
Lung Neoplasms
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
Lung Neoplasms
Cystic fibrosis transmembrane conductance regulator gene mutation and lung cancer risk.
Lung Neoplasms
Nicotine Induces Progressive Properties of Lung Adenocarcinoma A549 Cells by Inhibiting Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Expression and Plasma Membrane Localization.
Lung Neoplasms
The cystic fibrosis transmembrane conductance regulator as a biomarker in non-small cell lung cancer.
Lung Neoplasms
[Impact of Cystic Fibrosis Transmembrane Conductance Regulator on Malignant? Properties of KRAS Mutant Lung Adenocarcinoma A549 Cells].
Lymphoma
Immunohistochemical Detection of Markers for Translational Studies of Lung Disease in Pigs and Humans.
Lymphoma, B-Cell
Immunohistochemical Detection of Markers for Translational Studies of Lung Disease in Pigs and Humans.
Lymphopenia
Idiopathic CD4+ T lymphocytopenia presenting as mycetoma in a patient with a mutation in the cystic fibrosis transmembrane regulator gene.
Malnutrition
Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis.
Malnutrition
Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth.
Malnutrition
The need for vigilance: the case of a false-negative newborn screen for cystic fibrosis.
Meconium Ileus
Clinically Applicable Procedure for Gene Delivery to Fetal Gut by Ultrasound-Guided Gastric Injection: Toward Prenatal Prevention of Early-Onset Intestinal Diseases.
Meconium Ileus
Clinically applicable procedure for gene delivery to fetal gut by ultrasound-guided gastric injection: toward prenatal prevention of early-onset intestinal diseases.
Meconium Ileus
Prevalence of meconium ileus marks the severity of mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene.
Melanoma
Efficiency of plasmid delivery and expression after lipid-mediated gene transfer to human cells in vitro.
Metabolic Syndrome
Clinical evaluation of the Nanoduct sweat test system in the diagnosis of cystic fibrosis after newborn screening.
Metabolic Syndrome
Clinical outcomes in infants with cystic fibrosis transmembrane conductance regulator (CFTR) related metabolic syndrome.
Metabolic Syndrome
Cystic fibrosis infant care challenges in diagnosis and management in the era of newborn screening.
Metabolic Syndrome
Refining the continuum of CFTR-associated disorders in the era of newborn screening.
Mite Infestations
CFTR-deficiency renders mice highly susceptible to cutaneous symptoms during mite infestation.
Mitral Valve Prolapse
Pulmonary nontuberculous mycobacterial disease: prospective study of a distinct preexisting syndrome.
Multiple Endocrine Neoplasia
Fluorescence energy transfer detection as a homogeneous DNA diagnostic method.
Multiple Endocrine Neoplasia Type 2a
Fluorescence energy transfer detection as a homogeneous DNA diagnostic method.
Multiple Myeloma
TRIM44 links the UPS to SQSTM1/p62-dependent aggrephagy and removing misfolded proteins.
Muscular Dystrophy, Duchenne
Carbon nanofiber-based multiplexed immunosensor for the detection of survival motor neuron 1, cystic fibrosis transmembrane conductance regulator and Duchenne Muscular Dystrophy proteins.
Muscular Dystrophy, Duchenne
Heterozygous Cystic Fibrosis Transmembrane Regulator Gene Missense Variants Are Associated With Worse Cardiac Function in Patients With Duchenne Muscular Dystrophy.
Muscular Dystrophy, Duchenne
Multiplexed detection of DOCK8, PGM3 and STAT3 proteins for the diagnosis of Hyper-Immunoglobulin E syndrome using gold nanoparticles-based immunosensor array platform.
Mycetoma
Idiopathic CD4+ T lymphocytopenia presenting as mycetoma in a patient with a mutation in the cystic fibrosis transmembrane regulator gene.
Myocardial Ischemia
The impacts of along-channel acupuncture on the protein expressions of the chloride channel of the rats with myocardial ischemia.
Nasal Polyps
Localization of cystic fibrosis transmembrane conductance regulator in epithelial cells of nasal polyps and postoperative polypoid mucosae.
Nasal Polyps
The cystic fibrosis conductance regulator gene exon sequence is normal in a patient with edematous eosinophilic nasal polyps.
Nasal Polyps
[Expressions of cystic fibrosis transmembrane conductance regulator in epithelial cells of nasal polyps and normal turbinate mucosae]
Nasopharyngeal Carcinoma
CFTR is a potential marker for nasopharyngeal carcinoma prognosis and metastasis.
Neoplasms
A novel role and mechanism of cystic fibrosis transmembrane conductance regulator in bisphenol A-induced prostate cancer.
Neoplasms
ABC gene expression profiles have clinical importance and possibly form a new hallmark of cancer.
Neoplasms
Adenovirus?mediated overexpression of cystic fibrosis transmembrane conductance regulator enhances invasiveness and motility of serous ovarian cancer cells.
Neoplasms
Association of cystic fibrosis transmembrane conductance regulator (CFTR) mutation/variant/haplotype and tumor necrosis factor (TNF) promoter polymorphism in hyperlipidemic pancreatitis.
Neoplasms
CFTR deltaF508 carrier status, risk of breast cancer before the age of 40 and histological grading in a population-based case-control study.
Neoplasms
CFTR Functions as a Tumor Suppressor and Is Regulated by DNA Methylation in Colorectal Cancer.
Neoplasms
CFTR is a potential marker for nasopharyngeal carcinoma prognosis and metastasis.
Neoplasms
Constitutive activation of nuclear factor ?B contributes to cystic fibrosis transmembrane conductance regulator expression and promotes human cervical cancer progression and poor prognosis.
Neoplasms
Cystic fibrosis conductance regulator, tumor necrosis factor, interferon alpha-10, interferon alpha-17, and interferon gamma genotyping as potential risk markers in pulmonary sarcoidosis pathogenesis in Greek patients.
Neoplasms
Cystic Fibrosis Transmembrane Conductance Regulator Attaches Tumor Suppressor PTEN to the Membrane and Promotes Anti Pseudomonas aeruginosa Immunity.
Neoplasms
Cystic fibrosis transmembrane conductance regulator modulates neurosecretory function in pulmonary neuroendocrine cell-related tumor cell line models.
Neoplasms
Cytokine-stimulated nitric oxide production inhibits adenylyl cyclase and cAMP-dependent secretion in cholangiocytes.
Neoplasms
Downregulation of CFTR promotes epithelial-to-mesenchymal transition and is associated with poor prognosis of breast cancer.
Neoplasms
Effects and interaction of dietary electrolyte balance and citric acid on the intestinal function of weaned piglets.
Neoplasms
Expression of voltage-gated potassium channels in human and mouse colonic carcinoma.
Neoplasms
Gene expression profiling of ATP-binding cassette (ABC) transporters as a predictor of the pathologic response to neoadjuvant chemotherapy in breast cancer patients.
Neoplasms
Genetics of chronic obstructive pulmonary disease, beyond a1-antitrypsin deficiency.
Neoplasms
High level of CFTR expression is associated with tumor aggression and knockdown of CFTR suppresses proliferation of ovarian cancer in vitro and in vivo.
Neoplasms
In vivo investigation of acidified pepsin exposure to porcine vocal fold epithelia.
Neoplasms
Knockdown of CFTR enhances sensitivity of prostate cancer cells to cisplatin via inhibition of autophagy.
Neoplasms
Methylation profile of the promoter CpG islands of 14 "drug-resistance" genes in hepatocellular carcinoma.
Neoplasms
NF-kappa B mediates up-regulation of CFTR gene expression in Calu-3 cells by interleukin-1beta.
Neoplasms
Nicotine Induces Progressive Properties of Lung Adenocarcinoma A549 Cells by Inhibiting Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Expression and Plasma Membrane Localization.
Neoplasms
Overexpression of cystic fibrosis transmembrane conductance regulator (CFTR) is associated with human cervical cancer malignancy, progression and prognosis.
Neoplasms
Pancreatic intraepithelial neoplasia is associated with chronic pancreatitis due to serine protease inhibitor kazal type 1 and cystic fibrosis transmembrane conductance regulator mutations.
Neoplasms
Predictive value of cystic fibrosis transmembrane conductance regulator (CFTR) in the diagnosis of gastric cancer.
Neoplasms
Safety of local delivery of low- and intermediate-dose adenovirus gene transfer vectors to individuals with a spectrum of morbid conditions.
Neoplasms
The Enigmatic Gut in Cystic Fibrosis: Linking Inflammation, Dysbiosis, and the Increased Risk of Malignancy.
Neoplasms
Therapeutically Targeting Tumor Necrosis Factor-?/Sphingosine-1-Phosphate Signaling Corrects Myogenic Reactivity in Subarachnoid Hemorrhage.
Neoplasms
TRIM44 links the UPS to SQSTM1/p62-dependent aggrephagy and removing misfolded proteins.
Neoplasms
Tumor necrosis factor modulation of expression of the cystic fibrosis transmembrane conductance regulator gene.
Neoplasms
Tumor necrosis factor-?-mediated downregulation of the cystic fibrosis transmembrane conductance regulator drives pathological sphingosine-1-phosphate signaling in a mouse model of heart failure.
Neoplasms
Upregulation of CFTR in patients with endometriosis and its involvement in NF?B-uPAR dependent cell migration.
Neoplasms
Varied clinical significance of ATP-binding cassette C sub-family members for lung adenocarcinoma.
Neoplasms
[Impact of Cystic Fibrosis Transmembrane Conductance Regulator on Malignant? Properties of KRAS Mutant Lung Adenocarcinoma A549 Cells].
Nervous System Malformations
Rare association between cystic fibrosis, Chiari I malformation, and hydrocephalus in a baby: a case report and review of the literature.
Neuroblastoma
Cystic fibrosis transmembrane conductance regulator protein expression in brain.
Neurodegenerative Diseases
Molecular basis of UG-rich RNA recognition by the human splicing factor TDP-43.
Neurofibromatosis 1
In NF1, CFTR, PER3, CARS and SYT7, alternatively included exons show higher conservation of surrounding intron sequences than constitutive exons.
Oligospermia
Mutation frequency of cystic fibrosis transmembrane regulator is not increased in oligozoospermic male candidates for intracytoplasmic sperm injection.
Oligospermia
Transmission of male infertility and intracytoplasmic sperm injection (mini-review).
Opioid-Induced Constipation
Mashiningan Improves Opioid-Induced Constipation in Rats by Activating Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel.
Opioid-Induced Constipation
Phenylquinoxalinone CFTR activator as potential prosecretory therapy for constipation.
Ovarian Hyperstimulation Syndrome
Estrogen-induced abnormally high cystic fibrosis transmembrane conductance regulator expression results in ovarian hyperstimulation syndrome.
Ovarian Neoplasms
Adenovirus?mediated overexpression of cystic fibrosis transmembrane conductance regulator enhances invasiveness and motility of serous ovarian cancer cells.
Paget Disease, Extramammary
Cystic fibrosis transmembrane conductance regulator is helpful in the distinction of extra-mammary Paget's disease from squamous cell carcinoma in situ (Bowen's disease).
Pancreatic Cyst
Cystic Fibrosis-Related Pancreatic Cysts Decrease in Size and Number Upon Treatment With Cystic Fibrosis Transmembrane Conductance Regulator Modulators.
Pancreatic Diseases
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations and risk for pancreatic adenocarcinoma.
Pancreatic Diseases
Cystic fibrosis transmembrane regulator mutations and pancreatic disease: closing the gap between genotype and phenotype.
Pancreatic Neoplasms
CFTR, SPINK1, PRSS1, and CTRC Mutations Are Not Associated With Pancreatic Cancer in German Patients.
Pancreatic Neoplasms
Cystic fibrosis transmembrane regulator (CFTR) DeltaF508 mutation and 5T allele in patients with chronic pancreatitis and exocrine pancreatic cancer. PANKRAS II Study Group.
Pancreatitis
A novel exon duplication of the cystic fibrosis transmembrane conductance regulator in a patient presenting with adult-onset recurrent pancreatitis.
Pancreatitis
A Patient With Pancreas Divisum, Recurrent Acute Pancreatitis, and Homozygosity for the Cystic Fibrosis Transmembrane Regulator-Associated Protein 5T Allele.
Pancreatitis
A proinflammatory, antiapoptotic phenotype underlies the susceptibility to acute pancreatitis in cystic fibrosis transmembrane regulator (-/-) mice.
Pancreatitis
A rare mutation in cystic fibrosis transmembrane conductance regulator gene in a recurrent pancreatitis patient without respiratory symptoms.
Pancreatitis
Acute pancreatitis in association with Campylobacter jejuni-associated diarrhea in a 15-year-old with CFTR mutations: is there a link?
Pancreatitis
Acute Recurrent and Chronic Pancreatitis as Initial Manifestations of Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders.
Pancreatitis
Alcohol disrupts levels and function of the cystic fibrosis transmembrane conductance regulator to promote development of pancreatitis.
Pancreatitis
Analysis of the entire coding region of the cystic fibrosis transmembrane regulator gene in idiopathic pancreatitis.
Pancreatitis
Association of cystic fibrosis transmembrane conductance regulator (CFTR) mutation/variant/haplotype and tumor necrosis factor (TNF) promoter polymorphism in hyperlipidemic pancreatitis.
Pancreatitis
Association of pancreas divisum and recurrent acute pancreatitis with the IVS8-5T-12TG allele of the CFTR gene and CFTR dysfunction.
Pancreatitis
CFTR gene mutations in pancreatitis: Frequency and clinical manifestations in an Austrian patient cohort.
Pancreatitis
Chronic pancreatitis associated with the p.G208A variant of PRSS1 gene in a European patient.
Pancreatitis
Connections Between Genetics and Clinical Data: Role of MCP-1, CFTR, and SPINK-1 in the Setting of Acute, Acute Recurrent, and Chronic Pancreatitis.
Pancreatitis
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in pancreatitis.
Pancreatitis
Cystic fibrosis transmembrane conductance regulator functional evaluations in a G542X+/- IVS8Tn:T7/9 patient with acute recurrent pancreatitis.
Pancreatitis
Cystic fibrosis transmembrane conductance regulator ion channel function testing in recurrent acute pancreatitis.
Pancreatitis
Cystic fibrosis transmembrane conductance regulator modulators reduce the risk of recurrent acute pancreatitis among adult patients with pancreas sufficient cystic fibrosis.
Pancreatitis
Early acute pancreatitis in a child with compound heterozygosis ?F508/R1438W/Y1032C cystic fibrosis: a case report.
Pancreatitis
Ethanol and its non-oxidative metabolites profoundly inhibit CFTR function in pancreatic epithelial cells which is prevented by ATP supplementation.
Pancreatitis
Genetic, epidemiological, and clinical aspects of hereditary pancreatitis: a population-based cohort study in Denmark.
Pancreatitis
Hereditary pancreatitis in Japan: a review of pancreatitis-associated gene mutations.
Pancreatitis
High frequency of cystic fibrosis transmembrane regulator mutation L997F in patients with recurrent idiopathic pancreatitis and in newborns with hypertrypsinemia.
Pancreatitis
Identification of CFTR, PRSS1, and SPINK1 mutations in 381 patients with pancreatitis.
Pancreatitis
Incidence, Prevalence, Etiology, and Prognosis of First-Time Chronic Pancreatitis in Young Patients: A Nationwide Cohort Study.
Pancreatitis
Multifactorial Genesis of Pancreatitis in Primary Hyperparathyroidism: Evidence for "Protective" (PRSS2) and "Destructive" (CTRC) Genetic Factors.
Pancreatitis
Mutations in the cystic fibrosis transmembrane regulator gene in patients with tropical calcific pancreatitis.
Pancreatitis
Mutations of the cystic fibrosis gene, but not cationic trypsinogen gene, are associated with recurrent or chronic idiopathic pancreatitis.
Pancreatitis
Quantification of CFTR splice variants in adults with disseminated bronchiectasis, using the TaqMan fluorogenic detection system.
Pancreatitis
R248G cystic fibrosis transmembrane conductance regulator mutation in three siblings presenting with recurrent acute pancreatitis and reproductive issues: a case series.
Pancreatitis
Regulation of CFTR Bicarbonate Channel Activity by WNK1: Implications for Pancreatitis and CFTR-Related Disorders.
Pancreatitis
Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis.
Pancreatitis
Targeted Next-Generation Sequencing Effectively Analyzed the Cystic Fibrosis Transmembrane Conductance Regulator Gene in Pancreatitis.
Pancreatitis
The cystic fibrosis transmembrane conductance regulator gene and ion channel function in patients with idiopathic pancreatitis.
Pancreatitis
The Importance of Aquaporin 1 in Pancreatitis and Its Relation to the CFTR Cl- Channel.
Pancreatitis, Chronic
A finger sweat chloride test for the detection of a high-risk group of chronic pancreatitis.
Pancreatitis, Chronic
A novel mutation in the cystic fibrosis transmembrane conductance regulator gene in an Indian patient with idiopathic chronic pancreatitis: a case report.
Pancreatitis, Chronic
Acute pancreatitis in association with Campylobacter jejuni-associated diarrhea in a 15-year-old with CFTR mutations: is there a link?
Pancreatitis, Chronic
Association between F508 deletion in CFTR and chronic pancreatitis risk.
Pancreatitis, Chronic
CFTR gene mutations in pancreatitis: Frequency and clinical manifestations in an Austrian patient cohort.
Pancreatitis, Chronic
CFTR, PRSS1 and SPINK1 mutations in the development of pancreatitis in Brazilian patients.
Pancreatitis, Chronic
CFTR, SPINK1, PRSS1, and CTRC Mutations Are Not Associated With Pancreatic Cancer in German Patients.
Pancreatitis, Chronic
Chronic pancreatitis associated with the p.G208A variant of PRSS1 gene in a European patient.
Pancreatitis, Chronic
Chronic pancreatitis: controversies in etiology, diagnosis and treatment.
Pancreatitis, Chronic
Clinical and radiological outcome of patients suffering from chronic pancreatitis associated with gene mutations.
Pancreatitis, Chronic
Complete cystic fibrosis transmembrane conductance regulator gene sequencing in patients with idiopathic chronic pancreatitis and controls.
Pancreatitis, Chronic
Connections Between Genetics and Clinical Data: Role of MCP-1, CFTR, and SPINK-1 in the Setting of Acute, Acute Recurrent, and Chronic Pancreatitis.
Pancreatitis, Chronic
Current insights into the pathogenesis of acute and chronic pancreatitis.
Pancreatitis, Chronic
Cystic fibrosis transmembrane regulator (CFTR) DeltaF508 mutation and 5T allele in patients with chronic pancreatitis and exocrine pancreatic cancer. PANKRAS II Study Group.
Pancreatitis, Chronic
Determination of the relative contribution of three genes-the cystic fibrosis transmembrane conductance regulator gene, the cationic trypsinogen gene, and the pancreatic secretory trypsin inhibitor gene-to the etiology of idiopathic chronic pancreatitis.
Pancreatitis, Chronic
Different CFTR mutational spectrum in alcoholic and idiopathic chronic pancreatitis?
Pancreatitis, Chronic
Functional characteristics of L1156F-CFTR associated with alcoholic chronic pancreatitis in Japanese.
Pancreatitis, Chronic
Germline mutations in CFTR and PSTI genes in chronic pancreatitis patients.
Pancreatitis, Chronic
Impact of cystic fibrosis transmembrane conductance regulator gene mutation on the occurrence of chronic pancreatitis in Japanese patients.
Pancreatitis, Chronic
Isolated idiopathic chronic pancreatitis associated with a compound heterozygosity for two mutations of the CFTR gene.
Pancreatitis, Chronic
IVS8-5T Allele of CFTR is the Risk Factor in Chronic Pancreatitis, Especially in Idiopathic Chronic Pancreatitis.
Pancreatitis, Chronic
Molecular understanding of chronic pancreatitis: a perspective on the future.
Pancreatitis, Chronic
Motion--genetic testing is useful in the diagnosis of nonhereditary pancreatic conditions: arguments against the motion.
Pancreatitis, Chronic
Motion--genetic testing is useful in the diagnosis of nonhereditary pancreatic conditions: arguments for the motion.
Pancreatitis, Chronic
Mutation analysis of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, the cationic trypsinogen (PRSS1) gene, and the serine protease inhibitor, Kazal type 1 (SPINK1) gene in patients with alcoholic chronic pancreatitis.
Pancreatitis, Chronic
Mutations of the cystic fibrosis gene in patients with chronic pancreatitis.
Pancreatitis, Chronic
Pancreatic intraepithelial neoplasia is associated with chronic pancreatitis due to serine protease inhibitor kazal type 1 and cystic fibrosis transmembrane conductance regulator mutations.
Pancreatitis, Chronic
Pancreatitis severity in mice with impaired CFTR function but pancreatic sufficiency is mediated via ductal and inflammatory cells-Not acinar cells.
Pancreatitis, Chronic
Polymorphism of cystic fibrosis gene in Japanese patients with chronic pancreatitis.
Pancreatitis, Chronic
Relevance of variants in serum antiproteinases for the course of chronic pancreatitis.
Pancreatitis, Chronic
Spectrum of mutations and variants/haplotypes of CFTR and genotype-phenotype correlation in idiopathic chronic pancreatitis and controls in Chinese by complete analysis.
Pancreatitis, Chronic
The Cystic Fibrosis Transmembrane Conductance Regulator 470 Met Allele Is Associated with an Increased Risk of Chronic Pancreatitis in Both Asian and Caucasian Populations: A Meta-Analysis.
Pancreatitis, Chronic
[Pancreatitis in cystic fibrosis: association with genotype and pancreatic status.]
Paramyxoviridae Infections
?-Fetoprotein Gene Delivery to the Nasal Epithelium of Nonhuman Primates by Human Parainfluenza Viral Vectors.
Persistent Infection
Adenosine regulation of cystic fibrosis transmembrane conductance regulator through prostenoids in airway epithelia.
Persistent Infection
Calcium Modulated Chloride Pathways Contribute to Chloride Flux in Murine CF-Affected Macrophages.
Persistent Infection
Hypertonic saline increases lung epithelial lining fluid glutathione and thiocyanate: two protective CFTR-dependent thiols against oxidative injury.
Persistent Infection
Inflammation and CFTR: might neutrophils be the key in cystic fibrosis?
Persistent Infection
Lack of cystic fibrosis transmembrane conductance regulator disrupts fetal airway development in pigs.
Persistent Infection
Serum inflammatory profiles in cystic fibrosis mice with and without Bordetella pseudohinzii infection.
Persistent Infection
Sinus hypoplasia in the cystic fibrosis rat resolves in the absence of chronic infection.
Persistent Infection
Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis.
Persistent Infection
Therapeutic peptides for the treatment of cystic fibrosis: Challenges and perspectives.
Pneumonia
Accumulation of ceramide in the trachea and intestine of cystic fibrosis mice causes inflammation and cell death.
Pneumonia
Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis.
Pneumonia
Association of cystic fibrosis transmembrane conductance regulator gene variants with acute lung injury in African American children with pneumonia.
Pneumonia
Association of polymorphisms in genes of factors involved in regulation of splicing of cystic fibrosis transmembrane conductance regulator mRNA with acute respiratory distress syndrome in children with pneumonia.
Pneumonia
Cystic fibrosis transmembrane conductance regulator regulates epithelial cell response to Aspergillus and resultant pulmonary inflammation.
Pneumonia
Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth.
Pneumonia
Reduced GM1 Ganglioside in CFTR-Deficient Human Airway Cells Results in Decreased ss1-integrin Signaling and Delayed Wound Repair.
Pneumonia
Role of IL-10 deficiency in excessive nuclear factor-kappaB activation and lung inflammation in cystic fibrosis transmembrane conductance regulator knockout mice.
Polycystic Kidney Diseases
ABSOLUTE CONFIGURATION AND BIOLOGICAL PROPERTIES OF ENANTIOMERS OF CFTR INHIBITOR BPO-27.
Polycystic Kidney Diseases
Activation of AMP-activated kinase as a strategy for managing autosomal dominant polycystic kidney disease.
Polycystic Kidney Diseases
Alpha-aminoazaheterocyclic-methylglyoxal adducts do not inhibit cystic fibrosis transmembrane conductance regulator chloride channel activity.
Polycystic Kidney Diseases
Basolateral chloride transporters in autosomal dominant polycystic kidney disease.
Polycystic Kidney Diseases
Benzopyrimido-pyrrolo-oxazine-dione (R)-BPO-27 Inhibits CFTR Chloride Channel Gating by Competition with ATP.
Polycystic Kidney Diseases
CF gene and cystic fibrosis transmembrane conductance regulator expression in autosomal dominant polycystic kidney disease.
Polycystic Kidney Diseases
Cystic fibrosis transmembrane conductance regulator chloride channel blockers: Pharmacological, biophysical and physiological relevance.
Polycystic Kidney Diseases
Identification of resveratrol oligomers as inhibitors of cystic fibrosis transmembrane conductance regulator by high-throughput screening of natural products from chinese medicinal plants.
Polycystic Kidney Diseases
Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth.
Polycystic Kidney Diseases
Inhibition of histone deacetylase 6 activity reduces cyst growth in polycystic kidney disease.
Polycystic Kidney Diseases
Lipid Peroxidation Drives Renal Cyst Growth In Vitro through Activation of TMEM16A.
Polycystic Kidney Diseases
Mechanism and application of metformin in kidney diseases: An update.
Polycystic Kidney Diseases
Nanomolar Potency Pyrimido-pyrrolo-quinoxalinedione CFTR Inhibitor Reduces Cyst Size in a Polycystic Kidney Disease Model.
Polycystic Kidney Diseases
Novel action of the chalcone isoliquiritigenin as a cystic fibrosis transmembrane conductance regulator (CFTR) inhibitor: potential therapy for cholera and polycystic kidney disease.
Polycystic Kidney Diseases
Novel Action of the Chalcone Isoliquiritigenin as a Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Inhibitor: Potential Therapy for Cholera and Polycystic Kidney Disease.
Polycystic Kidney Diseases
Potent, Metabolically Stable Benzopyrimido-pyrrolo-oxazine-dione (BPO) CFTR Inhibitors for Polycystic Kidney Disease.
Polycystic Kidney Diseases
Small-molecule CFTR inhibitors slow cyst growth in polycystic kidney disease.
Polycystic Kidney Diseases
Steviol Reduces MDCK Cyst Formation and Growth by Inhibiting CFTR Channel Activity and Promoting Proteasome-Mediated CFTR Degradation.
Polycystic Kidney Diseases
Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease.
Polycystic Kidney Diseases
The cystic fibrosis transmembrane conductance regulator mediates transepithelial fluid secretion by human autosomal dominant polycystic kidney disease epithelium in vitro.
Polycystic Kidney Diseases
The relationship between cell proliferation, Cl- secretion, and renal cyst growth: a study using CFTR inhibitors.
Polycystic Kidney Diseases
The zebrafish Kupffer's vesicle as a model system for the molecular mechanisms by which the lack of Polycystin-2 leads to stimulation of CFTR.
Polycystic Kidney Diseases
Therapeutic potential of cystic fibrosis transmembrane conductance regulator (CFTR) inhibitors in polycystic kidney disease.
Polycystic Kidney, Autosomal Dominant
A regulatory role of polycystin-1 on cystic fibrosis transmembrane conductance regulator plasma membrane expression.
Polycystic Kidney, Autosomal Dominant
A role for CFTR in human autosomal dominant polycystic kidney disease.
Polycystic Kidney, Autosomal Dominant
Activation of AMP-activated kinase as a strategy for managing autosomal dominant polycystic kidney disease.
Polycystic Kidney, Autosomal Dominant
Autosomal dominant polycystic kidney disease coexisting with cystic fibrosis.
Polycystic Kidney, Autosomal Dominant
Basolateral chloride transporters in autosomal dominant polycystic kidney disease.
Polycystic Kidney, Autosomal Dominant
cAMP regulates cell proliferation and cyst formation in autosomal polycystic kidney disease cells.
Polycystic Kidney, Autosomal Dominant
CF gene and cystic fibrosis transmembrane conductance regulator expression in autosomal dominant polycystic kidney disease.
Polycystic Kidney, Autosomal Dominant
Exome sequencing of Saudi Arabian patients with ADPKD.
Polycystic Kidney, Autosomal Dominant
Fluid transport and cystogenesis in autosomal dominant polycystic kidney disease.
Polycystic Kidney, Autosomal Dominant
Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth.
Polycystic Kidney, Autosomal Dominant
Inhibition of histone deacetylase 6 activity reduces cyst growth in polycystic kidney disease.
Polycystic Kidney, Autosomal Dominant
Mechanism and application of metformin in kidney diseases: An update.
Polycystic Kidney, Autosomal Dominant
Role of CFTR in autosomal recessive polycystic kidney disease.
Polycystic Kidney, Autosomal Dominant
Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease.
Polycystic Kidney, Autosomal Dominant
Targeting chloride transport in autosomal dominant polycystic kidney disease.
Polycystic Kidney, Autosomal Dominant
The cystic fibrosis transmembrane conductance regulator mediates transepithelial fluid secretion by human autosomal dominant polycystic kidney disease epithelium in vitro.
Polycystic Kidney, Autosomal Dominant
The zebrafish Kupffer's vesicle as a model system for the molecular mechanisms by which the lack of Polycystin-2 leads to stimulation of CFTR.
Polycystic Kidney, Autosomal Dominant
Therapeutic potential of cystic fibrosis transmembrane conductance regulator (CFTR) inhibitors in polycystic kidney disease.
Polycystic Kidney, Autosomal Dominant
[Inhibitors of intra-cystic secretion in autosomal dominant polycystic kidney disease (ADPKD): a possible way forward].
Polycystic Ovary Syndrome
Impaired CFTR-Dependent Amplification of FSH-Stimulated Estrogen Production in Cystic Fibrosis and PCOS.
Prostatic Neoplasms
A novel role and mechanism of cystic fibrosis transmembrane conductance regulator in bisphenol A-induced prostate cancer.
Prostatic Neoplasms
Cystic fibrosis transmembrane conductance regulator (CFTR) gene 5T allele may protect against prostate cancer: a case-control study in Chinese Han population.
Prostatic Neoplasms
Knockdown of CFTR enhances sensitivity of prostate cancer cells to cisplatin via inhibition of autophagy.
Prostatic Neoplasms
Potential role of CFTR in bisphenol A-induced malignant transformation of prostate cells via mitochondrial apoptosis.
Pseudomonas Infections
16alpha-Bromoepiandrosterone (HE2000) limits non-productive inflammation and stimulates immunity in lungs.
Pseudomonas Infections
Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator.
Pseudomonas Infections
CFTR mutations and host susceptibility to Pseudomonas aeruginosa lung infection.
Pseudomonas Infections
Cystic fibrosis transmembrane conductance regulator (CFTR)-mediated residual chloride secretion does not protect against early chronic Pseudomonas aeruginosa infection in F508del homozygous cystic fibrosis patients.
Pseudomonas Infections
Influence of cystic fibrosis transmembrane conductance regulator on gene expression in response to Pseudomonas aeruginosa infection of human bronchial epithelial cells.
Pseudomonas Infections
Mucociliary clearance in cystic fibrosis knockout mice infected with Pseudomonas aeruginosa.
Pseudomonas Infections
Pathophysiology and management of pulmonary infections in cystic fibrosis.
Pseudomonas Infections
Role for cystic fibrosis transmembrane conductance regulator protein in a glutathione response to bronchopulmonary pseudomonas infection.
Pseudomonas Infections
Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections.
Pseudomonas Infections
Specific resistance to Pseudomonas aeruginosa infection in zebrafish is mediated by the cystic fibrosis transmembrane conductance regulator.
Pseudomonas Infections
Variants in the glutamate-cysteine-ligase gene are associated with cystic fibrosis lung disease.
Pulmonary Disease, Chronic Obstructive
Acquired defects in CFTR-dependent ?-adrenergic sweat secretion in chronic obstructive pulmonary disease.
Pulmonary Disease, Chronic Obstructive
Assessing effects of inhaled antibiotics in adults with non-cystic fibrosis bronchiectasis--experiences from recent clinical trials.
Pulmonary Disease, Chronic Obstructive
Augmentation of S-Nitrosoglutathione Controls Cigarette Smoke-Induced Inflammatory-Oxidative Stress and Chronic Obstructive Pulmonary Disease-Emphysema Pathogenesis by Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function.
Pulmonary Disease, Chronic Obstructive
Autophagy and inflammation in chronic respiratory disease.
Pulmonary Disease, Chronic Obstructive
CFTR dysfunction in cystic fibrosis and chronic obstructive pulmonary disease.
Pulmonary Disease, Chronic Obstructive
CFTR gene mutations--including three novel nucleotide substitutions--and haplotype background in patients with asthma, disseminated bronchiectasis and chronic obstructive pulmonary disease.
Pulmonary Disease, Chronic Obstructive
Cigarette Smoke-Induced Acquired Dysfunction of Cystic Fibrosis Transmembrane Conductance Regulator in the Pathogenesis of Chronic Obstructive Pulmonary Disease.
Pulmonary Disease, Chronic Obstructive
Correlation of apical fluid-regulating channel proteins with lung function in human COPD lungs.
Pulmonary Disease, Chronic Obstructive
Cystic Fibrosis Transmembrane Conductance Regulator. Implications in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Pulmonary Disease, Chronic Obstructive
Discovery of Icenticaftor (QBW251), a Cystic Fibrosis Transmembrane Conductance Regulator Potentiator with Clinical Efficacy in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Pulmonary Disease, Chronic Obstructive
Effect of lentivirus-mediated CFTR overexpression on oxidative stress injury and inflammatory response in the lung tissue of COPD mouse model.
Pulmonary Disease, Chronic Obstructive
Genetic and biochemical markers of obstructive lung disease in the general population.
Pulmonary Disease, Chronic Obstructive
Sequence analysis of the cystic fibrosis gene in patients with disseminated bronchiectatic lung disease. Application in the identification of a cystic fibrosis patient with atypical clinical course.
Pulmonary Disease, Chronic Obstructive
TGF-beta 1 and Cigarette Smoke Inhibit the Ability of ?2-agonists to Enhance Epithelial Permeability.
Pulmonary Disease, Chronic Obstructive
The mucus bundles responsible for airway cleaning are retained in cystic fibrosis and by cholinergic stimulation.
Pulmonary Disease, Chronic Obstructive
Unplugging Mucus in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Pulmonary Edema
Cystic Fibrosis Transmembrane Conductance Regulator Potentiation as a Therapeutic Strategy for Pulmonary Edema: A Proof-of-Concept Study in Pigs.
Pulmonary Edema
Cytokine-Regulation of Na(+)-K(+)-Cl(-) Cotransporter 1 and Cystic Fibrosis Transmembrane Conductance Regulator-Potential Role in Pulmonary Inflammation and Edema Formation.
Pulmonary Edema
Interdependency of beta-adrenergic receptors and CFTR in regulation of alveolar active Na+ transport.
Pulmonary Emphysema
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
Renal Insufficiency
Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease.
Respiratory Distress Syndrome
Association of polymorphisms in genes of factors involved in regulation of splicing of cystic fibrosis transmembrane conductance regulator mRNA with acute respiratory distress syndrome in children with pneumonia.
Respiratory Insufficiency
Mitochondrial oxidative stress in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice.
Respiratory Tract Infections
CFTR Heterozygotes Are at Increased Risk of Respiratory Infections: A Population-Based Study.
Respiratory Tract Infections
Regulation of the Coxsackie and adenovirus receptor expression is dependent on cystic fibrosis transmembrane regulator in airway epithelial cells.
Respiratory Tract Infections
The lactoperoxidase system links anion transport to host defense in cystic fibrosis.
Retinoschisis
Understanding human disease mutations through the use of interspecific genetic variation.
Rhinitis, Allergic
Effects of antiallergic herbal agents on cystic fibrosis transmembrane conductance regulator in nasal mucosal epithelia of allergic rhinitis rabbits.
Sarcoidosis
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
Sarcoidosis
Cystic fibrosis conductance regulator, tumor necrosis factor, interferon alpha-10, interferon alpha-17, and interferon gamma genotyping as potential risk markers in pulmonary sarcoidosis pathogenesis in Greek patients.
Sarcoidosis, Pulmonary
Cystic fibrosis conductance regulator, tumor necrosis factor, interferon alpha-10, interferon alpha-17, and interferon gamma genotyping as potential risk markers in pulmonary sarcoidosis pathogenesis in Greek patients.
Sarcoma
Differential effects of Matrigel and its components on functional activity of CFTR and ENaC in mouse endometrial epithelial cells.
Sarcoma, Avian
Adenovirus-mediated augmentation of cell transfection with unmodified plasmid vectors.
Sarcoma, Avian
Mechanism of enhancement of DNA expression consequent to cointernalization of a replication-deficient adenovirus and unmodified plasmid DNA.
Scoliosis
Pulmonary nontuberculous mycobacterial disease: prospective study of a distinct preexisting syndrome.
Sinusitis
Abnormal expression of the cystic fibrosis transmembrane regulator in chronic sinusitis in cystic fibrosis and non-cystic fibrosis patients.
Sinusitis
Cystic fibrosis gene mutations deltaF508 and 394delTT in patients with chronic sinusitis in Finland.
Sinusitis
Effect of L-ascorbate on chloride transport in freshly excised sinonasal epithelia.
Sinusitis
Maxillary sinusitis as a surrogate model for CF gene therapy clinical trials in patients with antrostomies.
Sinusitis
Multi-dimensional clinical phenotyping of a national cohort of adult cystic fibrosis patients.
Solitary Kidney
Cystic fibrosis transmembrane conductance regulator (CFTR) gene abnormalities in Indian males with congenital bilateral absence of vas deferens & renal anomalies.
Stomach Neoplasms
Heterogeneity in mouse spasmolytic polypeptide-expressing metaplasia lineages identifies markers of metaplastic progression.
Stomach Neoplasms
Predictive value of cystic fibrosis transmembrane conductance regulator (CFTR) in the diagnosis of gastric cancer.
T-Lymphocytopenia, Idiopathic CD4-Positive
Idiopathic CD4+ T lymphocytopenia presenting as mycetoma in a patient with a mutation in the cystic fibrosis transmembrane regulator gene.
Tangier Disease
Inhibitors of ATP-binding cassette transporters suppress interleukin-12 p40 production and major histocompatibility complex II up-regulation in macrophages.
Typhoid Fever
Structural basis of typhoid: Salmonella typhi type IVb pilin (PilS) and cystic fibrosis transmembrane conductance regulator interaction.
Typhoid Fever
Susceptibility to typhoid fever is associated with a polymorphism in the cystic fibrosis transmembrane conductance regulator (CFTR).
Uterine Cervical Neoplasms
CFTR Regulates the Proliferation, Migration and Invasion of Cervical Cancer Cells by Inhibiting the NF-?B Signalling Pathway.
Uterine Cervical Neoplasms
Constitutive activation of nuclear factor ?B contributes to cystic fibrosis transmembrane conductance regulator expression and promotes human cervical cancer progression and poor prognosis.
Uterine Cervical Neoplasms
Overexpression of cystic fibrosis transmembrane conductance regulator (CFTR) is associated with human cervical cancer malignancy, progression and prognosis.
Virus Diseases
Influenza virus M2 targets cystic fibrosis transmembrane conductance regulator for lysosomal degradation during viral infection.
Whooping Cough
G-protein regulation of outwardly rectified epithelial chloride channels incorporated into planar bilayer membranes.
Whooping Cough
Mobilization of intracellular Ca2+ and stimulation of cyclic AMP production by kappa opioid receptors expressed in Xenopus oocytes.
Whooping Cough
Role of pertussis toxin-sensitive G-proteins in intracellular Ca2+ release and apoptosis induced by inhibiting cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channels in HepG2 human hepatoblastoma cells.
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