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Disease on EC 5.6.1.6 - channel-conductance-controlling ATPase

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Abscess
CFTR Depletion Confers Hypersusceptibility to Mycobacterium fortuitum in a Zebrafish Model.
Acidosis
Cystic fibrosis-style changes in the early phase of pancreatitis.
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.
Cystic fibrosis transmembrane conductance regulator ameliorates lipopolysaccharide-induced acute lung injury by inhibiting autophagy through PI3K/AKT/mTOR pathway in mice.
Cystic fibrosis transmembrane conductance regulator modulates acute lung injury: evidence from a genetic association study*.
Novel role for cystic fibrosis transmembrane conductance regulator in alveolar fluid clearance in lipopolysaccharide-induced acute lung injury in mice.
Adenocarcinoma
cAMP-independent regulation of CFTR by the actin cytoskeleton.
Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations and risk for pancreatic adenocarcinoma.
Cystic fibrosis transmembrane regulator gene carrier status is a risk factor for young onset pancreatic adenocarcinoma.
Delta F508 CFTR localizes in the endoplasmic reticulum-Golgi intermediate compartment in cystic fibrosis cells.
MUC4 expression is regulated by cystic fibrosis transmembrane conductance regulator in pancreatic adenocarcinoma cells via transcriptional and post-translational mechanisms.
Nicotine Induces Progressive Properties of Lung Adenocarcinoma A549 Cells by Inhibiting Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Expression and Plasma Membrane Localization.
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
Anion secretion by a model epithelium: more lessons from Calu-3.
CFTR in Calu-3 human airway cells: channel properties and role in cAMP-activated Cl- conductance.
Nicotine Induces Progressive Properties of Lung Adenocarcinoma A549 Cells by Inhibiting Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Expression and Plasma Membrane Localization.
[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.
Lubiprostone Targets Prostanoid Signaling and Promotes Ion Transporter Trafficking, Mucus Exocytosis, and Contractility.
Adenoviridae Infections
Adenovirus-mediated in vivo gene transfer.
Adrenoleukodystrophy
[PMP70, the 70-kDa peroxisomal membrane protein: a member of the ATP-binding cassette transporters]
Airway Obstruction
Airway mucus in cystic fibrosis.
Assisting PNA transport through cystic fibrosis human airway epithelia with biodegradable hybrid lipid-polymer nanoparticles.
Cystic Fibrosis Transmembrane Conductance Regulator Function in Airway Smooth Muscle. A Novel Role in Cystic Fibrosis Airway Obstruction.
Inflammation and its genesis in cystic fibrosis.
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.
Frequency of cystic fibrosis transmembrane conductance regulator gene mutations and 5T allele in patients with allergic bronchopulmonary aspergillosis.
Link between CFTR mutations and ABPA: a systematic review and meta-analysis.
Asthma
Aerosolized Medications for Gene and Peptide Therapy.
Association between Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations and Susceptibility for Childhood Asthma in Korea.
Association of S549N and IVS8-5T Splice Variants with Bronchial Asthma and Its Severity in Indian Children.
Association of the CFTR gene with asthma and airway mucus hypersecretion.
Asthma and COPD in cystic fibrosis intron-8 5T carriers. A population-based study.
Asthma and pulmonary function abnormalities in heterozygotes for cystic fibrosis transmembrane regulator gene mutations.
CFTR gene mutations and asthma in the Norwegian Environment and Childhood Asthma study.
CFTR gene mutations--including three novel nucleotide substitutions--and haplotype background in patients with asthma, disseminated bronchiectasis and chronic obstructive pulmonary disease.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Asians with chronic pulmonary disease: a pilot study.
Cystic fibrosis transmembrane conductance regulator gene abnormalities in patients with asthma and recurrent neutrophilic bronchitis.
Genetic and biochemical markers of obstructive lung disease in the general population.
Missense mutations in the cystic fibrosis gene in adult patients with asthma.
Role of the cystic fibrosis transmembrane conductance channel in human airway smooth muscle.
Autoimmune Pancreatitis
Chronic 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.
A novel mutation (-195C>A) in the promoter region of CFTR gene is associated with Chinese Congenital Bilateral Absence of Vas Deferens (CBAVD).
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
Analysis of cystic fibrosis transmembrane regulator and azoospermia factor polymorphisms in infertile men in relation to other abnormalities.
Andrological findings in infertile men with two (biallelic) CFTR mutations: results of a multicentre study in Germany and Austria comprising 71 patients.
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 transmembrane conductance regulator and obstructive azoospermia.
Cytogenetic analysis of azoospermic patients: karyotype comparison of peripheral blood lymphocytes and testicular tissue.
Different cystic fibrosis transmembrane conductance regulator mutations in chinese men with congenital bilateral absence of vas deferens and other acquired obstructive azoospermia.
Genetic concerns for the subfertile male in the era of ICSI.
Genetic diseases of the seminal ducts.
Genetic markers of male infertility: Y chromosome microdeletions and cystic fibrosis transmembrane conductance gene mutations.
Improved detection of cystic fibrosis mutations in infertility patients with DNA sequence analysis.
Novel cause of hereditary obstructive azoospermia: a T2 allele in the CFTR gene.
Prevalence of CBAVD in azoospermic men carrying pathogenic CFTR mutations - Evaluated in a cohort of 639 non-vasectomized azoospermic men.
Proportion of cystic fibrosis gene mutations not detected by routine testing in men with obstructive 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).
Screening of ?F508 mutation and IVS8-poly T polymorphism in CFTR gene in Tunisian infertile men without CBAVD.
Seminal plasma characteristics as indicators of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in men with obstructive azoospermia.
The CFTR polymorphisms poly-T, TG-repeats and M470V in Chinese males with congenital bilateral absence of the vas deferens.
Transmission of male infertility and intracytoplasmic sperm injection (mini-review).
Truncating Mutations in the Adhesion G Protein-Coupled Receptor G2 Gene ADGRG2 Cause an X-Linked Congenital Bilateral Absence of Vas Deferens.
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.
Current concepts of immune dysregulation in cystic fibrosis.
Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function.
Dysfunctional CFTR alters the bactericidal activity of human macrophages against Pseudomonas aeruginosa.
Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice.
IL-17 primes airway epithelial cells lacking functional Cystic Fibrosis Transmembrane conductance Regulator (CFTR) to increase NOD1 responses.
Inflammation and its genesis in cystic fibrosis.
Inflammation in cystic fibrosis airways: relationship to increased bacterial adherence.
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.
Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis.
Progress toward generating a ferret model of cystic fibrosis by somatic cell nuclear transfer.
Resveratrol and ivacaftor are additive G551D CFTR-channel potentiators: therapeutic implications for cystic fibrosis sinus disease.
Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice.
The Application of Bicarbonate Recovers the Chemical-Physical Properties of Airway Surface Liquid in Cystic Fibrosis Epithelia Models.
The CFTR and ENaC debate: how important is ENaC in CF lung disease?
Tyrosine kinase c-Src constitutes a bridge between cystic fibrosis transmembrane regulator channel failure and MUC1 overexpression in cystic fibrosis.
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.
Cystic fibrosis and bone disease: defective osteoblast maturation with the f508del mutation in cystic fibrosis transmembrane conductance regulator.
Persistent osteopenia in adult cystic fibrosis transmembrane conductance regulator-deficient mice.
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.
Osteopenia in Cftr-deltaF508 mice.
Breast Neoplasms
Promoter hypermethylation of the CFTR gene as a novel diagnostic and prognostic marker of breast cancer.
[MDR (multidrug resistance) in hepatocarcinoma clinical-therapeutic implications]
Bronchiectasis
Analysis of CFTR Gene Variants in Idiopathic Bronchiectasis in Serbian Children.
Assessing effects of inhaled antibiotics in adults with non-cystic fibrosis bronchiectasis--experiences from recent clinical trials.
CFTR gene mutations--including three novel nucleotide substitutions--and haplotype background in patients with asthma, disseminated bronchiectasis and chronic obstructive pulmonary disease.
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Asians with chronic pulmonary disease: a pilot study.
Cystic Fibrosis Transmembrane Conductance Regulator Channel Dysfunction in Non-cystic Fibrosis Bronchiectasis.
Genetic risk factors for chronic obstructive pulmonary disease.
Increased frequency of cystic fibrosis deltaF508 mutation in bronchiectasis associated with rheumatoid arthritis.
Multi-dimensional clinical phenotyping of a national cohort of adult cystic fibrosis patients.
Novel cftr gene sequence variation in serbian patient with idiopathic disseminated bronchiectasis.
Progression of early structural lung disease in young children with cystic fibrosis assessed using CT.
Prospective analysis of cystic fibrosis transmembrane regulator mutations in adults with bronchiectasis or pulmonary nontuberculous mycobacterial infection.
Quantification of CFTR splice variants in adults with disseminated bronchiectasis, using the TaqMan fluorogenic detection system.
Recent advances in cystic fibrosis.
Role of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in patients with chronic sinopulmonary disease.
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.
Cigarette Smoke-Induced Acquired Dysfunction of Cystic Fibrosis Transmembrane Conductance Regulator in the Pathogenesis of Chronic Obstructive Pulmonary Disease.
Cigarette Smoke-induced Ca2+ Release Leads to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Dysfunction.
Common CFTR mutations are not likely to predispose to chronic bronchitis in northern Germany.
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
Cystic fibrosis transmembrane conductance regulator activation by roflumilast contributes to therapeutic benefit in chronic bronchitis.
Detection of CFTR gene mutations in patients suffering from chronic bronchitis.
Hydrator Therapies for Chronic Bronchitis. Lessons from Cystic Fibrosis.
Phosphodiesterase-4 Inhibitors for Non-COPD Respiratory Diseases.
Roflumilast reverses CFTR-mediated ion transport dysfunction in cigarette smoke-exposed mice.
TGF-beta 1 and Cigarette Smoke Inhibit the Ability of ?2-agonists to Enhance Epithelial Permeability.
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.
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.
CFTR Functions as a Tumor Suppressor and Is Regulated by DNA Methylation in Colorectal Cancer.
Characterization of the cAMP response element of the cystic fibrosis transmembrane conductance regulator gene promoter.
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).
Effects of mutations in cAMP-dependent protein kinase on chloride efflux in Caco-2 human colonic carcinoma cells.
Expression and Role of CFTR in Human Esophageal Squamous Cell Carcinoma.
Induction of expression of the cystic fibrosis transmembrane conductance regulator.
NF-kappaB activation is involved in regulation of cystic fibrosis transmembrane conductance regulator (CFTR) by interleukin-1beta.
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.
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.
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.
Cystic fibrosis transmembrane conductance regulator (CFTR) and autophagy: hereditary defects in cystic fibrosis versus gluten-mediated inhibition in celiac disease.
Solute transporters and aquaporins are impaired in celiac disease.
channel-conductance-controlling atpase deficiency
Acquired Cystic Fibrosis Transmembrane Conductance Regulator Deficiency.
Clinical practice and genetic counseling for cystic fibrosis and CFTR-related disorders.
Cystic fibrosis transmembrane conductance regulator deficiency exacerbates islet cell dysfunction after beta-cell injury.
Defects in Gallbladder Emptying and Bile Acid Homeostasis in Mice with Cystic Fibrosis Transmembrane Conductance Regulator Deficiencies.
Diet-Induced Dysbiosis and Genetic Background Synergize With Cystic Fibrosis Transmembrane Conductance Regulator Deficiency to Promote Cholangiopathy in Mice.
Self-assembled peptide-poloxamine nanoparticles enable in vitro and in vivo genome restoration for cystic fibrosis.
Transcriptional adaptation to cystic fibrosis transmembrane conductance regulator deficiency.
Cholangitis, Sclerosing
CFTR dysfunction predisposes to fibrotic liver disease in a murine model.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene defects in patients with primary sclerosing cholangitis.
Cystic fibrosis transmembrane conductance regulator gene polymorphisms in patients with primary sclerosing cholangitis.
Cytokine-stimulated nitric oxide production inhibits adenylyl cyclase and cAMP-dependent secretion in cholangiocytes.
Decreased Peroxisome Proliferator Activated Receptor alpha Is Associated with Bile Duct Injury in Cystic Fibrosis Transmembrane Conductance Regulator-/- Mice.
Lack of association of common cystic fibrosis transmembrane conductance regulator gene mutations with primary sclerosing cholangitis.
New insights into autoimmune cholangitis through animal models.
Novel association of HLA-haplotypes with primary sclerosing cholangitis (PSC) in a southern European population.
Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases.
Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function.
Survey of cystic fibrosis transmembrane conductance regulator genotypes in primary sclerosing cholangitis.
Cholelithiasis
New advances in acute pancreatitis.
Cholera
AC6 is the major adenylate cyclase forming a diarrheagenic protein complex with cystic fibrosis transmembrane conductance regulator in cholera.
Activation of AMP-activated protein kinase by a plant-derived dihydroisosteviol in human intestinal epithelial cell.
Adenylyl cyclase 6 expression is essential for cholera toxin-induced diarrhea.
An elusive adenylate cyclase complicit in cholera is exposed.
CFTR Inhibitors for Treating Diarrheal Disease.
CFTR Inhibitors.
Identification of new small molecule inhibitors of cystic fibrosis transmembrane conductance regulator protein: in vitro and in vivo studies.
Lectin conjugates as potent, nonabsorbable CFTR inhibitors for reducing intestinal fluid secretion in cholera.
Luminally active, nonabsorbable CFTR inhibitors as potential therapy to reduce intestinal fluid loss in cholera.
Lysophosphatidic acid inhibits cholera toxin-induced secretory diarrhea through CFTR-dependent protein interactions.
Mutual Enhancement of Virulence by Enterotoxigenic and Enteropathogenic Escherichia coli.
Nanomolar CFTR inhibition by pore-occluding divalent polyethylene glycol-malonic acid hydrazides.
New drug targets for cholera therapy.
Novel action of the chalcone isoliquiritigenin as a cystic fibrosis transmembrane conductance regulator (CFTR) inhibitor: potential therapy for cholera and polycystic kidney disease.
Novel Action of the Chalcone Isoliquiritigenin as a Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Inhibitor: Potential Therapy for Cholera and Polycystic Kidney Disease.
Shank2 Mutant Mice Display a Hypersecretory Response to Cholera Toxin.
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 in Benign Recurrent Intrahepatic Cholestasis 2.
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.
What is the evidence for genetics in chronic rhinosinusitis?
Coinfection
Unique microbial communities persist in individual cystic fibrosis patients throughout a clinical exacerbation.
Colitis
CFTR dysfunction predisposes to fibrotic liver disease in a murine model.
Expression of voltage-gated potassium channels in human and mouse colonic carcinoma.
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.
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.
Colorectal Neoplasms
Cystic Fibrosis, CFTR, and Colorectal Cancer.
Role of ion channels in gastrointestinal cancer.
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.
Nutritional status and eating disorders: neglected risks factor for nontuberculous mycobacterial lung disease?
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.
Genetic Polymorphisms in the Host and COVID-19 Infection.
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.
"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.
"Summer hypokalemia" as an initial presentation of cystic fibrosis in a morbidly obese African American adult: case report.
'CFTR-opathies': disease phenotypes associated with cystic fibrosis transmembrane regulator gene mutations.
1-BENZYLSPIRO[PIPERIDINE-4,1'-PYRIDO[3,4-b]indole] 'co-potentiators' for minimal function CFTR mutants.
150 mM HCO3(-)--how does the pancreas do it? Clues from computer modelling of the duct cell.
16alpha-Bromoepiandrosterone (HE2000) limits non-productive inflammation and stimulates immunity in lungs.
17beta-estradiol potentiates the cardiac cystic fibrosis transmembrane conductance regulator chloride current in guinea-pig ventricular myocytes.
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.
2-(dialkylamino)-4H-1-benzopyran-4-one derivatives modify chloride conductance in CFTR expressing cells.
3-(2-Benzyloxyphenyl)isoxazoles and isoxazolines: synthesis and evaluation as CFTR activators.
394delTT: a Nordic cystic fibrosis mutation.
40 kilobase deletion (CF 40 kb del 4-10) removes exons 4 to 10 of the Cystic Fibrosis Transmembrane Conductance Regulator gene.
5'-adenosine monophosphate mediated cooling treatment enhances ?F508-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) stability in vivo.
5'-Adenylylimidodiphosphate does not activate CFTR chloride channels in cell-free patches of membrane.
5-Hydroxytryptamine contributes significantly to a reflex pathway by which the duodenal mucosa protects itself from gastric acid injury.
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.
8-iso-PGE2 stimulates anion efflux from airway epithelial cells via the EP4 prostanoid receptor.
9-Phenanthrol inhibits human TRPM4 but not TRPM5 cationic channels.
? potential changing nanoparticles as cystic fibrosis transmembrane conductance regulator gene delivery system: an in vitro evaluation.
?-eudesmol but not atractylodin exerts an inhibitory effect on CFTR-mediated chloride transport in human intestinal epithelial cells.
?-Fetoprotein Gene Delivery to the Nasal Epithelium of Nonhuman Primates by Human Parainfluenza Viral Vectors.
?1 Syntrophin Supports Autophagy Initiation and Protects against Cerulein-Induced Acute Pancreatitis.
?2 adrenoceptor signaling regulates ion transport in 16HBE14o- human airway epithelial cells.
?2-Adrenoreceptor Agonist Inhalation During Ex Vivo Lung Perfusion Attenuates Lung Injury.
???-ENaC is inhibited by CFTR but stimulated by cAMP in Xenopus laevis oocytes.
?F508 CFTR interactome remodelling promotes rescue of cystic fibrosis.
?F508 CFTR surface stability is regulated by DAB2 and CHIP-mediated ubiquitination in post-endocytic compartments.
?F508-CFTR correctors: synthesis and evaluation of thiazole-tethered imidazolones, oxazoles, oxadiazoles, and thiadiazoles.
?F508-CFTR Modulator Screen Based on Cell Surface Targeting of a Chimeric Nucleotide Binding Domain 1 Reporter.
A 32-bp deletion (2991del32) in the cystic fibrosis gene associated with CFTR mRNA reduction.
A 96-well formatted method for exon and exon/intron boundary full sequencing of the CFTR gene.
A BAC Transgene Expressing Human CFTR under Control of Its Regulatory Elements Rescues Cftr Knockout Mice.
A balance between activating and repressive histone modifications regulates cystic fibrosis transmembrane conductance regulator (CFTR) expression in vivo.
A Bifidobacterium probiotic strain and its soluble factors alleviate chloride secretion by human intestinal epithelial cells.
A bird eye view on cystic fibrosis: An underestimated multifaceted chronic disorder.
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.
A CA repeat in the first intron of the CFTR gene.
A case report of CUAVD with azoospermia: a proposal of a rational diagnostic approach.
A Cdc48p-associated factor modulates endoplasmic reticulum-associated degradation, cell stress, and ubiquitinated protein homeostasis.
A CFTR chloride channel activator prevents HrpN(ea)-induced cell death in Arabidopsis thaliana suspension cells.
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.
A chemical corrector modifies the channel function of F508del-CFTR.
A chimeric type 2 adenovirus vector with a type 17 fiber enhances gene transfer to human airway epithelia.
A chloride channel widely expressed in epithelial and non-epithelial cells.
A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis.
A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein.
A cluster of highly polymorphic dinucleotide repeats in intron 17b of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
A cluster of negative charges at the amino terminal tail of CFTR regulates ATP-dependent channel gating.
A combination therapy for cystic fibrosis.
A combined analysis of the cystic fibrosis transmembrane conductance regulator: implications for structure and disease models.
A common mechanism for CFTR potentiators.
A common mechanism for cystic fibrosis transmembrane conductance regulator protein activation by genistein and benzimidazolone analogs.
A comparative genomic analysis of the cow, pig, and human CFTR genes identifies potential intronic regulatory elements.
A comparison of 14 antibodies for the biochemical detection of the cystic fibrosis transmembrane conductance regulator protein.
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.
A comparison of the performance of voltammetric aptasensors for glycated haemoglobin on different carbon nanomaterials-modified screen printed electrodes.
A complex intronic enhancer regulates expression of the CFTR gene by direct interaction with the promoter.
A comprehensive analysis of aquaporin and secretory related gene expression in neonate and adult cholangiocytes.
A conditional probability analysis of cystic fibrosis transmembrane conductance regulator gating indicates that ATP has multiple effects during the gating cycle.
A conserved region of the R domain of cystic fibrosis transmembrane conductance regulator is important in processing and function.
A controlled study of adenoviral-vector-mediated gene transfer in the nasal epithelium of patients with cystic fibrosis.
A cross-species analysis of the cystic fibrosis transmembrane conductance regulator. Potential functional domains and regulatory sites.
A current review of the safety of cystic fibrosis transmembrane conductance regulator modulators.
A cyclic nucleotide PDE5 inhibitor corrects defective mucin secretion in submandibular cells containing antibody directed against the cystic fibrosis transmembrane conductance regulator protein.
A cystic fibrosis allele encoding missense mutations in both nucleotide binding folds of the cystic fibrosis transmembrane conductance regulator.
A cystic fibrosis bronchial epithelial cell line: immortalization by adeno-12-SV40 infection.
A cystic fibrosis mutation associated with mild lung disease.
A cystic fibrosis transmembrane conductance regulator splice variant with partial penetrance associated with variable cystic fibrosis presentations.
A deletion mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) locus: Delta I507.
A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo.
A divergent CFTR homologue: highly regulated salt transport in the euryhaline teleost F. heteroclitus.
A domain mimic increases DeltaF508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia.
A finger sweat chloride test for the detection of a high-risk group of chronic pancreatitis.
A fixation method for the optimisation of western blotting.
A forskolin-activated Cl- current in mouse mandibular duct cells.
A functional CFTR assay using primary cystic fibrosis intestinal organoids.
A functional CFTR protein is required for mouse intestinal cAMP-, cGMP- and Ca(2+)-dependent HCO3- secretion.
A functional CFTR-NBF1 is required for ROMK2-CFTR interaction.
A functional R domain from cystic fibrosis transmembrane conductance regulator is predominantly unstructured in solution.
A Genome-Wide Analysis of Open Chromatin in Human Epididymis Epithelial Cells Reveals Candidate Regulatory Elements for Genes Coordinating Epididymal Function.
A Genotypic-Oriented View of CFTR Genetics Highlights Specific Mutational Patterns Underlying Clinical Macrocategories of Cystic Fibrosis.
A Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expression.
A haplotype framework for cystic fibrosis mutations in iran.
A haplotype-based molecular analysis of CFTR mutations associated with respiratory and pancreatic diseases.
A heteromeric complex of the two nucleotide binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) mediates ATPase activity.
A hierarchy of signals regulates entry of membrane proteins into the ciliary membrane domain in epithelial cells.
A High Level of Soluble CD40L Is Associated with P. aeruginosa Infection in Patients with Cystic Fibrosis.
A high prevalence of genetic polymorphisms in idiopathic and alcohol-associated chronic pancreatitis patients in Ireland.
A Homogeneous Cell-Based Halide-Sensitive Yellow Fluorescence Protein Assay to Identify Modulators of the Cystic Fibrosis Transmembrane Conductance Regulator Ion Channel.
A homogeneous method for genotyping with fluorescence polarization.
A human ciliopathy reveals essential functions for NEK10 in airway mucociliary clearance.
A kinase-regulated mechanism controls CFTR channel gating by disrupting bivalent PDZ domain interactions.
A large deletion causes apparent homozygosity for the D1152H mutation in the cystic fibrosis transmembrane regulator (CFTR) gene.
A large deletion in the CFTR gene in CBAVD.
A Link Between a Common Mutation in CFTR and Impaired Innate and Adaptive Viral Defense.
A macromolecular complex of beta 2 adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phosphoprotein 50 is regulated by PKA.
A method for measuring Cl efflux from dispersed cells of airway epithelium.
A method for the amplification of unknown flanking DNA: targeted inverted repeat amplification.
A microRNA network regulates expression and biosynthesis of wild-type and {Delta}F508 mutant cystic fibrosis transmembrane conductance regulator.
A missense cystic fibrosis transmembrane conductance regulator mutation with variable phenotype.
A missense mutation (F87L) in exon 3 of the cystic fibrosis transmembrane conductance regulator gene.
A missense mutation in SLC26A3 is associated with human male subfertility and impaired activation of CFTR.
A model-based economic evaluation of four newborn screening strategies for cystic fibrosis in Flanders, Belgium.
A Modified Alderman-Grant Coil makes possible an efficient cross-coil probe for high field solid-state NMR of lossy biological samples.
A molecular mechanism for aberrant CFTR-dependent HCO(3)(-) transport in cystic fibrosis.
A monoclonal antibody prevents aggregation of the NBD1 domain of the cystic fibrosis transmembrane conductance regulator.
A monomer is the minimum functional unit required for channel and ATPase activity of the cystic fibrosis transmembrane conductance regulator.
A mouse model for the cystic fibrosis delta F508 mutation.
A mouse model for the delta F508 allele of cystic fibrosis.
A murine model of cystic fibrosis.
A murine tracheal culture system to investigate parameters affecting gene therapy for cystic fibrosis.
A mutation in CFTR modifies the effects of the adenylate kinase inhibitor Ap5A on channel gating.
A mutation in the cystic fibrosis transmembrane conductance regulator gene associated with elevated sweat chloride concentrations in the absence of cystic fibrosis.
A mutation in the cystic fibrosis transmembrane conductance regulator generates a novel internalization sequence and enhances endocytic rates.
A mutation of the epithelial sodium channel associated with atypical cystic fibrosis increases channel open probability and reduces Na+ self inhibition.
A natural plant-derived dihydroisosteviol prevents cholera toxin-induced intestinal fluid secretion.
A naturally occurring sequence variation that creates a YY1 element is associated with increased cystic fibrosis transmembrane conductance regulator gene expression.
A neutral variant involved in a complex CFTR allele contributes to a severe cystic fibrosis phenotype.
A Neutralizing Aptamer to TGFBR2 and miR-145 Antagonism Rescue Cigarette Smoke- and TGF-?-Mediated CFTR Expression.
A new 9-alkyladenine-cyclic methylglyoxal diadduct activates wt- and F508del-cystic fibrosis transmembrane conductance regulator (CFTR) in vitro and in vivo.
A new approach for identifying non-pathogenic mutations. An analysis of the cystic fibrosis transmembrane regulator gene in normal individuals.
A new complex allele of the CFTR gene partially explains the variable phenotype of the L997F mutation.
A new compound heterozygous CFTR mutation in a Chinese family with cystic fibrosis.
A new era for people with cystic fibrosis.
A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect.
A new frameshift mutation 460delG in exon 4 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
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.
A new missense mutation (R1283M) in exon 20 of the cystic fibrosis transmembrane conductance regulator gene.
A new missense mutation G1249E in exon 20 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
A new missense mutation G126D in exon 4 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
A new model for fish ion regulation: identification of ionocytes in freshwater- and seawater-acclimated medaka (Oryzias latipes).
A new model of cystic fibrosis pathology: lack of transport of glutathione and its thiocyanate conjugates.
A new polymorphism in exon 7 of the cystic fibrosis transmembrane regulator (CFTR) gene.
A new role for bicarbonate secretion in cervico-uterine mucus release.
A new role for heat shock factor 27 in the pathophysiology of Clostridium difficile toxin B.
A new stochastic tridimensional model of neonatal rat spinal motoneuron for investigating compartmentalization of neuronal conductances and their influence on firing.
A New Targeted CFTR Mutation Panel Based on Next-Generation Sequencing Technology.
A nonolfactory shark adenosine receptor activates CFTR with unique pharmacology and structural features.
A nonsense mutation (R1158X) and a splicing mutation (3849 + 4A----G) in exon 19 of the cystic fibrosis transmembrane conductance regulator gene.
A Novel Case of Diabetic Muscle Necrosis in a Patient with Cystic Fibrosis-Related Diabetes.
A novel CFTR mutation found in a Chinese patient with cystic fibrosis.
A novel CFTR mutation, 4035delA, detected by non-radioactive SSCP analysis.
A Novel Chimeric Adenoassociated Virus 2/Human Bocavirus 1 Parvovirus Vector Efficiently Transduces Human Airway Epithelia.
A Novel Cystic Fibrosis Gene Mutation C.4242+1G>C in an Omani Patient: A Case Report.
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.
A novel ER J-protein DNAJB12 accelerates ER-associated degradation of membrane proteins including CFTR.
A novel exon duplication of the cystic fibrosis transmembrane conductance regulator in a patient presenting with adult-onset recurrent pancreatitis.
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.
A novel extract SB-300 from the stem bark latex of Croton lechleri inhibits CFTR-mediated chloride secretion in human colonic epithelial cells.
A novel fluorescent sensor for measurement of CFTR function by flow cytometry.
A Novel G542X CFTR Rat Model of Cystic Fibrosis Is Sensitive to Nonsense Mediated Decay.
A novel hemizygous loss-of-function mutation in ADGRG2 causes male infertility with congenital bilateral absence of the vas deferens.
A Novel in situ Approach to Studying Pancreatic Ducts in Mice.
A novel mammalian ER-located J-protein, DNAJB14, can accelerate ERAD of misfolded membrane proteins.
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.
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.
A novel missense mutation in exon 16 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene identified in CBAVD patients.
A novel missense mutation P1290S at exon-20 of the CFTR gene in a Portuguese patient with congenital bilateral absence of the vas deferens.
A Novel Missense Mutation, R1283S, of the Cystic Fibrosis Transmembrane Conductance Regulator Gene in a 47-Year-Old African-American Patient.
A novel model for the first nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator.
A novel mutation (-195C>A) in the promoter region of CFTR gene is associated with Chinese Congenital Bilateral Absence of Vas Deferens (CBAVD).
A novel mutation (M1V) in the translation initiation codon of the cystic fibrosis transmembrane conductance regulator gene, in three CF chromosomes of Italian origin.
A novel mutation detected by temporal temperature gradient gel electrophoresis led to the confirmative prenatal diagnosis of a Hispanic CF family.
A novel mutation in exon 3 of the CFTR gene.
A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations.
A novel mutation in the cystic fibrosis transmembrane conductance regulator gene in an Indian patient with idiopathic chronic pancreatitis: a case report.
A novel natural product compound enhances cAMP-regulated chloride conductance of cells expressing CFTR[delta]F508.
A novel nonsense mutation, S434X, in exon 9 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
A novel nonsense mutation, S466Xa in exon 10 of the cystic fibrosis transmembrane conductance regulator gene.
A novel nonsense mutation, W846XI (amber termination), in exon 14a of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
A novel PDZ protein regulates the activity of guanylyl cyclase C, the heat-stable enterotoxin receptor.
A novel procedure for the efficient purification of the cystic fibrosis transmembrane conductance regulator (CFTR).
A novel quality control compartment derived from the endoplasmic reticulum.
A novel regulatory role for tissue transglutaminase in epithelial-mesenchymal transition in cystic fibrosis.
A novel role and mechanism of cystic fibrosis transmembrane conductance regulator in bisphenol A-induced prostate cancer.
A novel role of protein tyrosine kinase2 in mediating chloride secretion in human airway epithelial cells.
A novel splice mutation, 4006-1G>A, in intron 21 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
A novel splice site mutation in the first exon of the cystic fibrosis transmembrane regulator (CFTR) gene identified in a CBAVD patient.
A novel transporter, Pfcrt, confers antimalarial drug resistance.
A novel treatment of cystic fibrosis acting on-target: cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR.
A nuclear factor that binds purine-rich, single-stranded oligonucleotides derived from S1-sensitive elements upstream of the CFTR gene and the MUC1 gene.
A pathogenic role for cystic fibrosis transmembrane conductance regulator in celiac disease.
A Patient With Pancreas Divisum, Recurrent Acute Pancreatitis, and Homozygosity for the Cystic Fibrosis Transmembrane Regulator-Associated Protein 5T Allele.
A PDZ-binding motif is essential but not sufficient to localize the C terminus of CFTR to the apical membrane.
A PDZ-interacting domain in CFTR is an apical membrane polarization signal.
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.
A Peptide Nucleic Acid against MicroRNA miR-145-5p Enhances the Expression of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Calu-3 Cells.
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.
A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease.
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.
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.
A phase I study of aerosolized administration of tgAAVCF to cystic fibrosis subjects with mild lung disease.
A phase II, double-blind, randomized, placebo-controlled clinical trial of tgAAVCF using maxillary sinus delivery in patients with cystic fibrosis with antrostomies.
A physiologically-motivated model of cystic fibrosis liquid and solute transport dynamics across primary human nasal epithelia.
A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function.
A pilot study of the effect of gentamicin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations.
A placebo-controlled study of liposome-mediated gene transfer to the nasal epithelium of patients with cystic fibrosis.
A Plant-Derived Hydrolysable Tannin Inhibits CFTR Chloride Channel: A Potential Treatment of Diarrhea.
A plant-derived hydrolysable tannin inhibits CFTR chloride channel: a potential treatment of diarrhea.
A plethora of cardiac chloride conductances: molecular diversity or a related gene family.
A possible role for intracellular GSH in spontaneous reaction of a cysteine (T338C) engineered into the Cystic Fibrosis Transmembrane Conductance Regulator.
A potential strategy for reducing cysts in autosomal dominant polycystic kidney disease with a CFTR corrector.
A potentiator induces conformational changes on the recombinant CFTR nucleotide binding domains in solution.
A primary culture model of differentiated murine tracheal epithelium.
A principal role for the proteasome in endoplasmic reticulum-associated degradation of misfolded intracellular cystic fibrosis transmembrane conductance regulator.
A proinflammatory, antiapoptotic phenotype underlies the susceptibility to acute pancreatitis in cystic fibrosis transmembrane regulator (-/-) mice.
A protein sequence that can encode native structure by disfavoring alternate conformations.
A quantitative multistandard reverse transcriptase-polymerase chain reaction assay of the cystic fibrosis transmembrane conductance regulator: its usefulness in studying efficiency of gene transfer.
A Randomized Clinical Trial of Metformin to Treat Autosomal Dominant Polycystic Kidney Disease.
A randomized placebo-controlled trial of miglustat in cystic fibrosis based on nasal potential difference.
A Rapid Membrane Potential Assay to Monitor CFTR Function and Inhibition.
A rapid, efficient, and sensitive assay for simultaneous detection of multiple cystic fibrosis mutations.
A Rare Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mutation Associated With Typical Cystic Fibrosis in an Arab Child.
A rare DNA variant in exon 15 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
A rare mutation in cystic fibrosis transmembrane conductance regulator gene in a recurrent pancreatitis patient without respiratory symptoms.
A rat parotid gland cell line, Par-C10, exhibits neurotransmitter-regulated transepithelial anion secretion.
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.
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.
A recombinant polypeptide model of the second predicted nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator is a GTP-binding protein.
A regulatory element in intron 1 of the cystic fibrosis transmembrane conductance regulator gene.
A regulatory role of polycystin-1 on cystic fibrosis transmembrane conductance regulator plasma membrane expression.
A release mechanism for stored ATP in ocular ciliary epithelial cells.
A Review of Trikafta: Triple Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulator Therapy.
A Review on the Use of Cystic Fibrosis Transmembrane Conductance Regulator Gene Modulators in Pediatric Patients.
A revised airway epithelial hierarchy includes CFTR-expressing ionocytes.
A role for CFTR in human autosomal dominant polycystic kidney disease.
A role for CFTR in the elevation of glutathione levels in the lung by oral glutathione administration.
A role for the cystic fibrosis transmembrane conductance regulator in the nitric oxide-dependent release of Cl(-) from acidic organelles in amacrine cells.
A safety evaluation of ivacaftor for the treatment of cystic fibrosis.
A SAXS-based ensemble model of the native and phosphorylated regulatory domain of the CFTR.
A Scientific Rationale for Using Cystic Fibrosis Transmembrane Conductance Regulator Therapeutics in COVID-19 Patients.
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.
A sensitive, real-time, RNA-specific PCR method for the detection of recombinant AAV-CFTR vector expression.
A sequence upstream of canonical PDZ-binding motif within CFTR COOH-terminus enhances NHERF1 interaction.
A sequence variation in intron 17B of the cystic fibrosis transmembrane conductance regulator gene.
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.
A simple, fast and inexpensive method for mutation scanning of CFTR gene.
A simplified cyclic adenosine monophosphate-mediated sweat rate test for quantitative measure of cystic fibrosis transmembrane regulator (CFTR) function.
A single amino acid substitution in CFTR converts ATP to an inhibitory ligand.
A single conductance pore for chloride ions formed by two cystic fibrosis transmembrane conductance regulator molecules.
A small molecule modulator interacts directly with deltaF508-CFTR to modify its ATPase activity and conformational stability.
A small molecule that binds to an ATPase domain of Hsc70 promotes membrane trafficking of mutant cystic fibrosis transmembrane conductance regulator.
A spatial model of fluid recycling in the airways of the lung.
A specific cystic fibrosis mutation (T3381) associated with the phenotype of isolated hypotonic dehydration.
A splicing mutation in intron 16 of the cystic fibrosis transmembrane conductance regulator gene, associated with severe disease, is common on Reunion Island.
A stable ATP binding to the nucleotide binding domain is important for reliable gating cycle in an ABC transporter CFTR.
A Stable Human-Cell System Overexpressing Cystic Fibrosis Transmembrane Conductance Regulator Recombinant Protein at the Cell Surface.
A study of aqueous humor formation in patients with cystic fibrosis.
A study of the mechanism of the release of ATP from rat cortical astroglial cells evoked by activation of glutamate receptors.
A survey of detergents for the purification of stable, active human cystic fibrosis transmembrane conductance regulator (CFTR).
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
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.
A SYK/SHC1 pathway regulates the amount of CFTR in the plasma membrane.
A synonymous codon change alters the drug sensitivity of ?F508 cystic fibrosis transmembrane conductance regulator.
A synonymous single nucleotide polymorphism in DeltaF508 CFTR alters the secondary structure of the mRNA and the expression of the mutant protein.
A synthetic channel-forming peptide induces Cl(-) secretion: modulation by Ca(2+)-dependent K(+) channels.
A synthetic chloride channel relaxes airway smooth muscle of the rat.
A synthetic chloride channel restores chloride conductance in human cystic fibrosis epithelial cells.
A Tannic Acid-Based Medical Food, Cesinex(®), Exhibits Broad-Spectrum Antidiarrheal Properties: A Mechanistic and Clinical Study.
A termination mutation (2143delT) in the CFTR gene of German cystic fibrosis patients.
A topological switch in CFTR modulates channel activity and sensitivity to unfolding.
A transcription factor network represses
A transformed human tracheal gland cell line, MM-39, that retains serous secretory functions.
A two-domain model for the R domain of the cystic fibrosis transmembrane conductance regulator based on sequence similarities.
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.
A unique subset of rat and human intestinal villus cells express the cystic fibrosis transmembrane conductance regulator.
A universal array-based multiplexed test for cystic fibrosis carrier screening.
A variable dinucleotide repeat in the CFTR gene contributes to phenotype diversity by forming RNA secondary structures that alter splicing.
A yeast artificial chromosome contig encompassing the cystic fibrosis locus.
A yeast metal resistance protein similar to human cystic fibrosis transmembrane conductance regulator (CFTR) and multidrug resistance-associated protein.
A yeast phenomic model for the gene interaction network modulating CFTR-?F508 protein biogenesis.
A(2) adenosine receptors regulate CFTR through PKA and PLA(2).
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.
A2 adenosine receptors in Mongolian gerbil middle ear epithelium and their regulation of Cl- secretion.
A2B adenosine receptors regulate the mucus clearance component of the lung's innate defense system.
AAV exploits subcellular stress associated with inflammation, endoplasmic reticulum expansion, and misfolded proteins in models of 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.
ABCA1: regulation, trafficking and association with heteromeric proteins.
ABCC7/CFTR Expression Is Associated with the Clinical Course of Ulcerative Colitis Patients.
Aberrant CFTR-dependent HCO3- transport in mutations associated with cystic fibrosis.
Aberrant GSH reductase and NOX activities concur with defective CFTR to pro-oxidative imbalance in cystic fibrosis airways.
Ability of adenovirus vectors containing different CFTR transcriptional cassettes to correct ion transport defects in CF cells.
Ablation of internalization signals in the carboxyl-terminal tail of the cystic fibrosis transmembrane conductance regulator enhances cell surface expression.
Abnormal enamel development in a cystic fibrosis transgenic mouse model.
Abnormal expression of the cystic fibrosis transmembrane regulator in chronic sinusitis in cystic fibrosis and non-cystic fibrosis patients.
Abnormal ion content, hydration and granule expansion of the secretory granules from cystic fibrosis airway glandular cells.
Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia.
Abnormal n-6 fatty acid metabolism in cystic fibrosis contributes to pulmonary symptoms.
Abnormal Rab11-Rab8-vesicles cluster in enterocytes of patients with microvillus inclusion disease.
Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes.
Abnormally enhanced cystic fibrosis transmembrane conductance regulator-mediated apoptosis in endometrial cells contributes to impaired embryo implantation in controlled ovarian hyperstimulation.
Abnormally up-regulated cystic fibrosis transmembrane conductance regulator expression and uterine fluid accumulation contribute to Chlamydia trachomatis-induced female infertility.
Abolition of Ca2+-mediated intestinal anion secretion and increased stool dehydration in mice lacking the intermediate conductance Ca2+-dependent K+ channel Kcnn4.
Absence of CFTR is associated with pleiotropic effects on mucins in mouse gallbladder epithelial cells.
Absence of the cystic fibrosis transmembrane regulator (Cftr) from myeloid-derived cells slows resolution of inflammation and infection.
Absence of typical unfolded protein response in primary cultured cystic fibrosis airway epithelial cells.
ABSOLUTE CONFIGURATION AND BIOLOGICAL PROPERTIES OF ENANTIOMERS OF CFTR INHIBITOR BPO-27.
Abundant off-target edits from site-directed RNA editing can be reduced by nuclear localization of the editing enzyme.
AC6 is the major adenylate cyclase forming a diarrheagenic protein complex with cystic fibrosis transmembrane conductance regulator in cholera.
Academy of Nutrition and Dietetics: 2020 Cystic Fibrosis Evidence Analysis Center Evidence-Based Nutrition Practice Guideline.
Acceleration of glutathione efflux and inhibition of gamma-glutamyltranspeptidase sensitize metastatic B16 melanoma cells to endothelium-induced cytotoxicity.
Accessory protein facilitated CFTR-CFTR interaction, a molecular mechanism to potentiate the chloride channel activity.
Accumulation of azithromycin and roxithromycin in tracheal epithelial fetal cell lines expressing wild type or mutated cystic fibrosis transmembrane conductance regulator protein (CFTR).
Accumulation of ceramide in the trachea and intestine of cystic fibrosis mice causes inflammation and cell death.
Accumulation of metals in GOLD4 COPD lungs is associated with decreased CFTR levels.
Acid and base secretion in the Calu-3 model of human serous cells.
Acid-stimulated duodenal bicarbonate secretion involves a CFTR-mediated transport pathway in mice.
Acidic Submucosal Gland pH and Elevated Protein Concentration Produce Abnormal Cystic Fibrosis Mucus.
Acidification-dependent activation of CD1d-restricted natural killer T cells is intact in cystic fibrosis.
Acquired Cystic Fibrosis Transmembrane Conductance Regulator Deficiency.
Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis and Other Diseases of Mucus Clearance.
Acquired cystic fibrosis transmembrane conductance regulator dysfunction.
Acquired defects in CFTR-dependent ?-adrenergic sweat secretion in chronic obstructive pulmonary disease.
Actin directly interacts with different membrane channel proteins and influences channel activities: AQP2 as a model.
Actin filament organization is required for proper cAMP-dependent activation of CFTR.
Action of neltenexine on anion secretion in human airway epithelia.
Actions of adenosine A1 and A2 receptor antagonists on CFTR antibody-inhibited beta-adrenergic mucin secretion response.
Actions of genistein on cystic fibrosis transmembrane conductance regulator channel gating. Evidence for two binding sites with opposite effects.
Activating AMP-activated protein kinase (AMPK) slows renal cystogenesis.
Activating cystic fibrosis transmembrane conductance regulator channels with pore blocker analogs.
Activation mechanisms for the cystic fibrosis transmembrane conductance regulator protein involve direct binding of cAMP.
Activation of a CFTR-mediated chloride current in a rabbit corneal epithelial cell line.
Activation of AMP-activated kinase as a strategy for managing autosomal dominant polycystic kidney disease.
Activation of AMP-activated protein kinase by a plant-derived dihydroisosteviol in human intestinal epithelial cell.
Activation of AMP-activated protein kinase reduces cAMP-mediated epithelial chloride secretion.
Activation of AMPK inhibits cholera toxin stimulated chloride secretion in human and murine intestine.
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.
Activation of apical CFTR and basolateral Ca(2+)-activated K+ channels by tetramethylpyrazine in Caco-2 cell line.
Activation of cAMP-dependent C1- currents in guinea-pig paneth cells without relevant evidence for CFTR expression.
Activation of cardiac chloride conductance by the tyrosine kinase inhibitor, genistein.
Activation of CFTR by ASBT-mediated bile salt absorption.
Activation of CFTR by genistein in human airway epithelial cell lines.
Activation of CFTR by the Flavonoid Quercetin: Potential Use as a Biomarker of {Delta}F508 CFTR Rescue.
Activation of CFTR chloride current by nitric oxide in human T lymphocytes.
Activation of CFTR Cl(-) channel by tyrphostins via a protein tyrosine kinase-independent pathway in forskolin-stimulated renal epithelial A6 cells.
Activation of CFTR trafficking and gating by vasoactive intestinal peptide in human bronchial epithelial cells.
Activation of Cl- currents by intracellular chloride in fibroblasts stably expressing the human cystic fibrosis transmembrane conductance regulator.
Activation of constitutive androstane receptor inhibits intestinal CFTR-mediated chloride transport.
Activation of cystic fibrosis transmembrane conductance regulator in rat epididymal epithelium by genistein.
Activation of deltaF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX.
Activation of DeltaF508 CFTR in an epithelial monolayer.
Activation of endogenous deltaF508 cystic fibrosis transmembrane conductance regulator by phosphodiesterase inhibition.
Activation of G551D CFTR channel with MPB-91: regulation by ATPase activity and phosphorylation.
Activation of intestinal Cl- secretion by lubiprostone requires the cystic fibrosis transmembrane conductance regulator.
Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells.
Activation of the cystic fibrosis transmembrane conductance regulator by cGMP in the human colonic cancer cell line, Caco-2.
Activation of the cystic fibrosis transmembrane regulator by cyclic AMP is not correlated with inhibition of endocytosis.
Activation of the unfolded protein response by DeltaF508 CFTR.
Activation of transgene expression by early region 4 is responsible for a high level of persistent transgene expression from adenovirus vectors in vivo.
Activation of wild-type and deltaF508-CFTR by phosphodiesterase inhibitors through cAMP-dependent and -independent mechanisms.
Active-Site Flexibility and Substrate Specificity in a Bacterial Virulence Factor: Crystallographic Snapshots of an Epoxide Hydrolase.
Activity and inhibition of prostasin and matriptase on apical and basolateral surfaces of human airway epithelial cells.
Acute appendicitis mimicking intestinal obstruction in a patient with cystic fibrosis.
Acute inflammation alters bicarbonate transport in mouse ileum.
Acute inhibition of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel by thyroid hormones involves multiple mechanisms.
Acute lung injury edema fluid decreases net fluid transport across human alveolar epithelial type II cells.
Acute pancreatitis in association with Campylobacter jejuni-associated diarrhea in a 15-year-old with CFTR mutations: is there a link?
Acute Pulmonary Exacerbations in Cystic Fibrosis.
Acute Recurrent and Chronic Pancreatitis as Initial Manifestations of Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders.
Acute regulation of the SLC26A3 congenital chloride diarrhoea anion exchanger (DRA) expressed in Xenopus oocytes.
Acute responses of non-human primates to airway delivery of an adenovirus vector containing the human cystic fibrosis transmembrane conductance regulator cDNA.
Acylpeptide hydrolase inhibition as targeted strategy to induce proteasomal down-regulation.
Adapting in situ polymerase chain reaction for genotyping of cells in suspension.
Adapting Proteostasis and Autophagy for Controlling the Pathogenesis of Cystic Fibrosis Lung Disease.
Additional disruption of the ClC-2 Cl(-) channel does not exacerbate the cystic fibrosis phenotype of cystic fibrosis transmembrane conductance regulator mouse models.
Adenine nucleotide-induced activation of adenosine A(2B) receptors expressed in Xenopus laevis oocytes: involvement of a rapid and localized adenosine formation by ectonucleotidases.
Adeno-associated virus (AAV)-mediated gene transfer in respiratory epithelium and submucosal gland cells in human fetal tracheal organ culture.
Adeno-associated virus for cystic fibrosis gene therapy.
Adenocarcinoma of the gastrointestinal tract in lung transplanted patients with cystic fibrosis: case series and review of the literature.
Adenosine activates a2b receptors and enhances chloride secretion in kidney inner medullary collecting duct cells.
Adenosine and its nucleotides activate wild-type and R117H CFTR through an A2B receptor-coupled pathway.
Adenosine inhibits arginine vasopressin-stimulated chloride secretion in a mouse IMCD cell line (mIMCD-K2).
Adenosine receptors and phosphodiesterase inhibitors stimulate Cl- secretion in Calu-3 cells.
Adenosine receptors in rat and human pancreatic ducts stimulate chloride transport.
Adenosine receptors, cystic fibrosis, and airway hydration.
Adenosine regulation of cystic fibrosis transmembrane conductance regulator through prostenoids in airway epithelia.
Adenosine triphosphate release and purinergic (P2) receptor-mediated secretion in small and large mouse cholangiocytes.
Adenosine triphosphate-binding cassette superfamily transporter gene expression in severe male infertility.
Adenosine triphosphate-dependent asymmetry of anion permeation in the cystic fibrosis transmembrane conductance regulator chloride channel.
Adenoviral vector expressing ICP47 inhibits adenovirus-specific cytotoxic T lymphocytes in nonhuman primates.
Adenovirus-mediated augmentation of cell transfection with unmodified plasmid vectors.
Adenovirus-mediated gene transfer to ciliated airway epithelia requires prolonged incubation time.
Adenovirus-mediated gene transfer transiently corrects the chloride transport defect in nasal epithelia of patients with cystic fibrosis.
Adenovirus-mediated in vivo gene transfer.
Adenovirus-mediated persistent cystic fibrosis transmembrane conductance regulator expression in mouse airway epithelium.
Adenovirus-mediated transfer of the CFTR gene to lung of nonhuman primates: toxicity study.
Adenovirus?mediated overexpression of cystic fibrosis transmembrane conductance regulator enhances invasiveness and motility of serous ovarian cancer cells.
Adenylate Cyclase 1 Links Calcium Signaling to CFTR-Dependent Cytosolic Chloride Elevations in Chick Amacrine Cells.
Adenylyl cyclase 6 expression is essential for cholera toxin-induced diarrhea.
Adherence to cystic fibrosis transmembrane conductance regulator (CFTR) modulators: analysis of a national specialty pharmacy database.
ADP inhibits function of the ABC transporter cystic fibrosis transmembrane conductance regulator via its adenylate kinase activity.
Adrenaline-induced colonic K+ secretion is mediated via KCa1.1 (BK) channel.
Adult Care in Cystic Fibrosis.
Advances in Cell and Gene-based Therapies for Cystic Fibrosis Lung Disease.
Advances in cystic fibrosis therapies.
Advances In Gene Therapy For Cystic Fibrosis Lung Disease.
Aerosol administration of a recombinant adenovirus expressing CFTR to cystic fibrosis patients: a phase I clinical trial.
Aerosol delivery of lipid:DNA complexes to lungs of rhesus monkeys.
Aerosol-mediated delivery of recombinant adenovirus to the airways of nonhuman primates.
Aerosolized Bovine Lactoferrin Counteracts Infection, Inflammation and Iron Dysbalance in A Cystic Fibrosis Mouse Model of Pseudomonas aeruginosa Chronic Lung Infection.
Aerosolized Medications for Gene and Peptide Therapy.
Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors.
Affinity purification of insoluble recombinant fusion proteins containing glutathione-S-transferase.
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.
After the Celebrations: Lessons from the New Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy.
Aggregation of misfolded proteins can be a selective process dependent upon peptide composition.
Aggresomes: a cellular response to misfolded proteins.
Agonist-induced coordinated trafficking of functionally related transport proteins for water and ions in cholangiocytes.
Agonists that stimulate secretion promote the recruitment of CFTR into membrane lipid microdomains.
Airway acidification initiates host defense abnormalities in cystic fibrosis mice.
Airway epithelial cell exposure to distinct e-cigarette liquid flavorings reveals toxicity thresholds and activation of CFTR by the chocolate flavoring 2,5-dimethypyrazine.
Airway epithelial cells-Functional links between CFTR and anoctamin dependent Cl(-) secretion.
Airway epithelial CFTR mRNA expression in cystic fibrosis patients after repetitive administration of a recombinant adenovirus.
Airway epithelial control of Pseudomonas aeruginosa infection in cystic fibrosis.
Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies.
Airway Epithelial Wounds in Rhesus Monkey Generate Ionic Currents That Guide Cell Migration to Promote Healing.
Airway epithelium directed gene therapy for cystic fibrosis.
Airway gene transfer in mouse nasal-airways: importance of identification of epithelial type for assessment of gene transfer.
Airway hydration and COPD.
Airway hyperresponsiveness in FVB/N delta F508 cystic fibrosis transmembrane conductance regulator mice.
Airway Inflammation and Host Responses in the Era of CFTR Modulators.
Airway inflammation and infection in congenital bilateral absence of the vas deferens.
Airway microbiota and pathogen abundance in age-stratified cystic fibrosis patients.
Airway microenvironment alterations and pathogen growth in cystic fibrosis.
Airway mucus in cystic fibrosis.
Airway nitric oxide levels in cystic fibrosis patients are related to a polymorphism in the neuronal nitric oxide synthase gene.
Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis.
Airway surface liquid composition in mice.
Airway surface liquid homeostasis in cystic fibrosis: pathophysiology and therapeutic targets.
Airway surface pH in subjects with cystic fibrosis.
Airways in cystic fibrosis are acidified: detection by exhaled breath condensate.
AKAP proteins anchor cAMP-dependent protein kinase to KvLQT1/IsK channel complex.
Alcohol disrupts levels and function of the cystic fibrosis transmembrane conductance regulator to promote development of pancreatitis.
Alcoholic pancreatitis and polymorphisms of the variable length polythymidine tract in the cystic fibrosis gene.
Alignment of transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Allele frequency for Cystic fibrosis in Indians vis-a/-vis global populations.
Allergic bronchopulmonary aspergillosis in asthma and cystic fibrosis.
Allergic Bronchopulmonary Aspergillosis in Children with Cystic Fibrosis: An Update on the Newest Diagnostic Tools and Therapeutic Approaches.
Alpha-1 Antitrypsin-A Target for MicroRNA-Based Therapeutic Development for Cystic Fibrosis.
Alpha-aminoazaheterocyclic-methylglyoxal adducts do not inhibit cystic fibrosis transmembrane conductance regulator chloride channel activity.
Alpha1-antitrypsin deficiency alleles and the Taq-I G-->A allele in cystic fibrosis lung disease.
ALTERATION IN THE CYTOKINE SECRETION PATTERN IN T CELLS OF PATIENTS WITH CYSTIC FIBROSIS CAUSED BY DNA METHYLTRANSFERASE INHIBITOR 5-AZACITIDINE.
Alteration of CFTR transmembrane span integration by disease-causing mutations.
Alteration of Membrane Cholesterol Content Plays a Key Role in Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Channel Activity.
Alteration of protein function by a silent polymorphism linked to tRNA abundance.
Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway.
Alterations of the Nasopharyngeal Microbiota in Infants with Cystic Fibrosis. Cystic Fibrosis Transmembrane Conductance Regulator and Antibiotic Effects.
Altered beta-adrenergic and muscarinic response of CFTR Cl- current in dialyzed cardiac myocytes.
Altered biogenesis of deltaF508-CFTR following treatment with doxorubicin.
Altered channel gating mechanism for CFTR inhibition by a high-affinity thiazolidinone blocker.
Altered cyclic expression of epithelial Na+ channel subunits and cystic fibrosis transmembrane conductance regulator in mouse endometrium by a low sodium diet.
Altered eicosanoid metabolism in the cystic fibrosis mouse small intestine.
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.
Altered ion transport by thyroid epithelia fromCFTR-/- pigs suggests mechanisms for hypothyroidism in Cystic Fibrosis.
Altered NaCl concentration of airway surface liquid in cystic fibrosis.
Altered pHi regulation in 3T3/CFTR clones and their chemotherapeutic drug-selected derivatives.
Altered protein folding may be the molecular basis of most cases of cystic fibrosis.
Altered respiratory epithelial cell cytokine production in cystic fibrosis.
Altered sulfate transport via anion exchange in CFPAC is corrected by retrovirus-mediated CFTR gene transfer.
Altering intracellular pH reveals the kinetic basis of intraburst gating in the CFTR Cl(-) channel.
Altering Metabolic Profiles of Drugs by Precision Deuteration 2: Discovery of a Deuterated Analog of Ivacaftor with Differentiated Pharmacokinetics for Clinical Development.
Alternate stimulation of apical CFTR by genistein in epithelia.
Alternate translation initiation codons can create functional forms of cystic fibrosis transmembrane conductance regulator.
Alternating access to the transmembrane domain of the ATP-binding cassette protein cystic fibrosis transmembrane conductance regulator (ABCC7).
Alternative 5' exons of the CFTR gene show developmental regulation.
Alternative splicing of a previously unidentified CFTR exon introduces an in-frame stop codon 5' of the R region.
Alternative splicing of CFTR Cl- channels in heart.
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.
Alveolar epithelium: role in lung fluid balance and acute lung injury.
Alveolar inflammation in cystic fibrosis.
Ambroxol-induced modification of ion transport in human airway Calu-3 epithelia.
Amiloride-sensitive fluid resorption in NCI-H441 lung epithelia depends on an apical Cl(-) conductance.
Amino acid residues lining the chloride channel of the cystic fibrosis transmembrane conductance regulator.
Amino acid substitution matrices from an information theoretic perspective.
Amino acid substitutions in the sixth transmembrane domain of P-glycoprotein alter multidrug resistance.
Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.
Ammonia exposure affects the mRNA and protein expression levels of certain Rhesus glycoproteins in the gills of climbing perch.
AMP-activated protein kinase and nitric oxide regulate the glucose sensitivity of ventromedial hypothalamic glucose-inhibited neurons.
AMPK Agonists Ameliorate Sodium and Fluid Transport and Inflammation in CF Airway Epithelial Cells.
Amplification of CFTR exon 9 sequences to multiple locations in the human genome.
Amplifiers co-translationally enhance CFTR biosynthesis via PCBP1-mediated regulation of CFTR mRNA.
Amplifying dinucleotide microsatellite loci from bone and tooth samples of up to 5000 years of age: more inconsistency than usefulness.
Amylase expression in taste receptor cells of rat circumvallate papillae.
An "ex vivo model" contributing to the diagnosis and evaluation of new drugs in cystic fibrosis.
An "Unlikely" Pair: The Antimicrobial Synergy of Polymyxin B in Combination with the Cystic Fibrosis Transmembrane Conductance Regulator Drugs KALYDECO and ORKAMBI.
An Adult Mouse Model of Vibrio cholerae-induced Diarrhea for Studying Pathogenesis and Potential Therapy of Cholera.
An alternate pathway of cAMP-stimulated Cl secretion across the NKCC1-null murine duodenum.
An Ancient CFTR Ortholog Informs Molecular Evolution in ABC Transporters.
An animal model for cystic fibrosis made by gene targeting.
An anion channel in guinea pig gallbladder epithelial cells is highly permeable to HCO(-)(3).
An apical PDZ protein anchors the cystic fibrosis transmembrane conductance regulator to the cytoskeleton.
An electrostatic interaction at the tetrahelix bundle promotes phosphorylation-dependent cystic fibrosis transmembrane conductance regulator (CFTR) channel opening.
An element in intron 1 of the CFTR gene augments intestinal expression in vivo.
An elusive adenylate cyclase complicit in cholera is exposed.
An energy-dependent maturation step is required for release of the cystic fibrosis transmembrane conductance regulator from early endoplasmic reticulum biosynthetic machinery.
An exonic splicing enhancer offsets the atypical GU-rich 3' splice site of human apolipoprotein A-II exon 3.
An image analysis method to quantify CFTR subcellular localization.
An improved method to obtain highly differentiated monolayers of human bronchial epithelial cells.
An Innovative Protocol for Metaproteomic Analyses of Microbial Pathogens in Cystic Fibrosis Sputum.
An integrated mathematical epithelial cell model for airway surface liquid regulation by mechanical forces.
An international randomized multicenter comparison of nasal potential difference techniques.
An Intriguing Involvement of Mitochondria in Cystic Fibrosis.
An intrinsic adenylate kinase activity regulates gating of the ABC transporter CFTR.
An intronic polypyrimidine-rich element downstream of the donor site modulates cystic fibrosis transmembrane conductance regulator exon 9 alternative splicing.
An inwardly rectifying potassium channel in apical membrane of Calu-3 cells.
An MBL2 haplotype and ABCB4 variants modulate the risk of liver disease in cystic fibrosis patients: a multicentre study.
An Optimised Human Cell Culture Model for Alveolar Epithelial Transport.
An overview of the pathogenesis of cystic fibrosis lung disease.
An ovine CFTR variant as a putative cystic fibrosis causing mutation.
An unexpected effect of TNF-α on F508del-CFTR maturation and function.
An unstable transmembrane segment in the cystic fibrosis transmembrane conductance regulator.
An update on new and emerging therapies for cystic fibrosis.
Analysis of 30 known cystic fibrosis mutations: 10 mutations account for 27% of non-delta F508 chromosomes in southern France.
Analysis of 31 CFTR mutations by polymerase chain reaction/oligonucleotide ligation assay in a pilot screening of 4476 newborns for cystic fibrosis.
Analysis of a DNase I hypersensitive site located -20.9 kb upstream of the CFTR gene.
Analysis of alternative splicing patterns in the cystic fibrosis transmembrane conductance regulator gene using mRNA derived from lymphoblastoid cells of cystic fibrosis patients.
Analysis of c.3499+200TA(7_56) and D7S523 Microsatellites Linked to Cystic Fibrosis Transmembrane Regulator.
Analysis of CA/GT microsatellite polymorphism in IVS8 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene: a study of Italian CF families.
Analysis of CFTR Gene Mutations in Children with Cystic Fibrosis, First Report from North-East of Iran.
Analysis of CFTR Gene Variants in Idiopathic Bronchiectasis in Serbian Children.
Analysis of CFTR interactome in the macromolecular complexes.
Analysis of cis and trans Requirements for DNA Replication at the Right-End Hairpin of the Human Bocavirus 1 Genome.
Analysis of Conventional and Unconventional Trafficking of CFTR and Other Membrane Proteins.
Analysis of cystic fibrosis transmembrane conductance regulator gene mutations in patients with congenital absence of the uterus and vagina.
Analysis of cystic fibrosis transmembrane regulator and azoospermia factor polymorphisms in infertile men in relation to other abnormalities.
Analysis of cystic fibrosis-associated P67L CFTR illustrates barriers to personalized therapeutics for orphan diseases.
Analysis of DNase-I-hypersensitive sites at the 3' end of the cystic fibrosis transmembrane conductance regulator gene (CFTR).
ANALYSIS OF LOCAL IMMUNITY INDICATORS OF THE ORAL CAVITY AND DEGREE OF GINGIVITIS DEPENDING ON MUTATION OF CFTR GENE IN CHILDREN WITH CYSTIC FIBROSIS.
Analysis of long-range interactions in primary human cells identifies cooperative CFTR regulatory elements.
Analysis of microsatellites by direct blotting electrophoresis and chemiluminescence detection.
Analysis of polymerase chain reaction-amplified DNA products by mass spectrometry using matrix-assisted laser desorption and electrospray: current status.
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.
Analysis of the CFTR gene confirms the high genetic heterogeneity of the Spanish population: 43 mutations account for only 78% of CF chromosomes.
Analysis of the CFTR gene in Iranian cystic fibrosis patients: identification of eight novel mutations.
Analysis of the CFTR gene in Turkish cystic fibrosis patients: identification of three novel mutations (3172delAC, P1013L and M1028I).
Analysis of the entire coding region of the cystic fibrosis transmembrane regulator gene in neonatal hypertrypsinaemia with normal sweat test.
Analysis of the entire coding region of the cystic fibrosis transmembrane regulator gene in idiopathic pancreatitis.
Analysis of the human pancreatic secretory trypsin inhibitor (PSTI) gene mutations in Japanese patients with chronic pancreatitis.
Analysis of the intron-exon organization of the human multidrug-resistance protein gene (MRP) and alternative splicing of its mRNA.
Analysis of the localization of STE6/CFTR chimeras in a Saccharomyces cerevisiae model for the cystic fibrosis defect CFTR delta F508.
Analysis of the mouse and rat CFTR promoter regions.
Analysis of the whole CFTR coding regions and splice junctions in azoospermic men with congenital bilateral aplasia of epididymis or vas deferens.
Analytical validation considerations of multiplex mass-spectrometry-based proteomic platforms for measuring protein biomarkers.
Anchored PDE4 regulates chloride conductance in wild-type and ?F508-CFTR human airway epithelia.
Andrological findings in infertile men with two (biallelic) CFTR mutations: results of a multicentre study in Germany and Austria comprising 71 patients.
Angiotensin II evokes calcium-mediated signaling events in isolated dog pancreatic epithelial cells.
Animal and model systems for studying cystic fibrosis.
Animal models for cystic fibrosis liver disease (CFLD).
Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences.
Anion conductance selectivity mechanism of the CFTR chloride channel.
Anion secretion by a model epithelium: more lessons from Calu-3.
Anion secretion by the inner medullary collecting duct. Evidence for involvement of the cystic fibrosis transmembrane conductance regulator.
Anion transport in heart.
Annexin A5 increases the cell surface expression and the chloride channel function of the DeltaF508-cystic fibrosis transmembrane regulator.
Annexin V is directly involved in cystic fibrosis transmembrane conductance regulator's chloride channel function.
Anoctamin 1 in secretory epithelia.
Anoctamin 1 induces calcium-activated chloride secretion and proliferation of renal cyst-forming epithelial cells.
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.
Anoctamin 6 is an essential component of the outwardly rectifying chloride channel.
Anoctamins support calcium-dependent chloride secretion by facilitating calcium signaling in adult mouse intestine.
ANP and CNP activate CFTR expressed in Xenopus laevis oocytes by direct activation of PKA.
Antagonistic Regulation of CFTR Cell Surface Expression by Protein Kinases WNK4 and Spleen Tyrosine Kinase.
Antagonists of myo-inositol 3,4,5,6-tetrakisphosphate allow repeated epithelial chloride secretion.
Anti-Infectives Restore ORKAMBI® Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of P. aeruginosa.
Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis.
Antibiotic exposure and interpersonal variance mask the effect of ivacaftor on respiratory microbiota composition.
Antibodies against the cystic fibrosis transmembrane regulator.
Antibodies for CFTR studies.
Antibodies to the CFTR modulate the turgor pressure of guard cell protoplasts via slow anion channels.
Antibody against a cystic fibrosis transmembrane conductance regulator-derived synthetic peptide inhibits anion currents in human colonic cell line T84.
Antidiarrheal Action of Bacillus subtilis CU1 CNCM I-2745 and Lactobacillus plantarum CNCM I-4547 in Mice.
Antidiarrheal efficacy of a quinazolin CFTR inhibitor on human intestinal epithelial cell and in mouse model of cholera.
Antihypertensive 1,4-dihydropyridines as correctors of the cystic fibrosis transmembrane conductance regulator channel gating defect caused by cystic fibrosis mutations.
Antimicrobial Properties of Mesenchymal Stem Cells: Therapeutic Potential for Cystic Fibrosis Infection, and Treatment.
Antineutrophil cytoplasmic autoantibodies (ANCA) in children with cystic fibrosis.
Antioxidant imbalance in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice.
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.
Antisense oligodeoxynucleotides to the cystic fibrosis transmembrane conductance regulator inhibit cAMP-activated but not calcium-activated chloride currents.
Antisense oligonucleotide eluforsen is safe and improves respiratory symptoms in F508DEL cystic fibrosis.
Antisense oligonucleotide to PKC-epsilon alters cAMP-dependent stimulation of CFTR in Calu-3 cells.
Antisense oligonucleotides to CFTR confer a cystic fibrosis phenotype on B lymphocytes.
Antisera preparation and epitope mapping of a recombinant protein comprising three peptide fragments of the cystic fibrosis transmembrane conductance regulator.
AP2 ? modulates cystic fibrosis transmembrane conductance regulator function in the human intestine.
Apical adenosine regulates basolateral Ca2+-activated potassium channels in human airway Calu-3 epithelial cells.
Apical and basolateral ATP stimulates tracheal epithelial chloride secretion via multiple purinergic receptors.
Apical heterotrimeric g-proteins activate CFTR in the native sweat duct.
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.
Apparent affinity of CFTR for ATP is increased by continuous kinase activity.
Apparent homozygosity of a novel frame shift mutation in the CFTR gene because of a large deletion.
Apparent Homozygosity of p.Phe508del in CFTR due to a Large Gene Deletion of Exons 4-11.
Applicability of different antibodies for immunohistochemical localization of CFTR in sweat glands from healthy controls and from patients with cystic fibrosis.
Application of DETECTER, an evolutionary genomic tool to analyze genetic variation, to the cystic fibrosis gene family.
Application of high-resolution single-channel recording to functional studies of cystic fibrosis mutants.
Application of mass spectrometry to study proteomics and interactomics in cystic fibrosis.
Application of pyrosequencing to the identification of sequence variations in the cystic fibrosis transmembrane conductance regulator gene.
Application of the luminex LabMAP in rapid screening for mutations in the cystic fibrosis transmembrane conductance regulator gene: A pilot study
Applying Cystic Fibrosis Transmembrane Conductance Regulator Genetics and CFTR2 Data to Facilitate Diagnoses.
Approach to the infertile man.
Aquagenic Wrinkling of the Palm: A Rare Diagnostic Clue of Cystic Fibrosis and the Response to CFTR-Modulating Therapy.
Aquagenic wrinkling of the palms in cystic fibrosis and the cystic fibrosis carrier state: a case–control study.
Aquaporin 3 cloned from Xenopus laevis is regulated by the cystic fibrosis transmembrane conductance regulator.
Aquaporin-4 as a molecular partner of cystic fibrosis transmembrane conductance regulator in rat Sertoli cells.
Aquaporin-9 is expressed in rat Sertoli cells and interacts with the cystic fibrosis transmembrane conductance regulator.
Aquaporins and CFTR in ocular epithelial fluid transport.
Aqueous cigarette smoke extract induces a voltage-dependent inhibition of CFTR expressed in Xenopus oocytes.
AR-13 reduces antibiotic-resistant bacterial burden in cystic fibrosis phagocytes and improves cystic fibrosis transmembrane conductance regulator function.
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.
Architectural proteins CTCF and cohesin have distinct roles in modulating the higher order structure and expression of the CFTR locus.
Architecture and functional properties of the CFTR channel pore.
Architecture of the cystic fibrosis transmembrane conductance regulator protein and structural changes associated with phosphorylation and nucleotide binding.
Arg352 is a major determinant of charge selectivity in the cystic fibrosis transmembrane conductance regulator chloride channel.
Arrhythmia and Sudden Death Associated with Elevated Cardiac Chloride Channel Activity.
Arsenic promotes ubiquitinylation and lysosomal degradation of cystic fibrosis transmembrane conductance regulator (CFTR) chloride channels in human airway epithelial cells.
Arsenite regulates Cystic Fibrosis Transmembrane Conductance Regulator and P-glycoprotein: evidence of pathway independence.
Arylaminobenzoate block of the cardiac cyclic AMP-dependent chloride current.
Aspergillus colonization and antifungal immunity in cystic fibrosis patients.
Aspergillus fumigatus generates an enhanced Th2-biased immune response in mice with defective cystic fibrosis transmembrane conductance regulator.
Aspergillus-induced superoxide production by cystic fibrosis phagocytes is associated with disease severity.
Aspirin and some other nonsteroidal anti-inflammatory drugs inhibit cystic fibrosis transmembrane conductance regulator protein gene expression in T-84 cells.
Assembly and Functional Analysis of an S/MAR Based Episome with the Cystic Fibrosis Transmembrane Conductance Regulator Gene.
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.
Assembly and trafficking of a multiprotein ROMK (Kir 1.1) channel complex by PDZ interactions.
Assembly of functional CFTR chloride channels.
Assembly of gap junction channels: mechanism, effects of calmodulin antagonists and identification of connexin oligomerization determinants.
Assembly of signaling complexes by the sodium-hydrogen exchanger regulatory factor family of PDZ-containing proteins.
Assessing effects of inhaled antibiotics in adults with non-cystic fibrosis bronchiectasis--experiences from recent clinical trials.
Assessing the Disease-Liability of Mutations in CFTR.
Assessment of acquired mucociliary clearance defects using micro-optical coherence tomography.
Assessment of Aspergillus sensitization or persistent carriage as a factor in lung function impairment in cystic fibrosis patients.
Assessment of body composition in pediatric patients with cystic fibrosis.
Assessment of CFTR function in rectal biopsies for the diagnosis of cystic fibrosis.
Assessment of chloride secretion in human nasal epithelial cells by X-ray microanalysis.
Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation.
Assessment of dental status and oral hygiene in the study population of cystic fibrosis patients in the Podlasie province.
Assessment of hospitalization rates, factors associated with hospitalization and in-patient mortality in pediatric patients with cystic fibrosis.
Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cells.
Assessment of the CFTR and ENaC association.
Assisted reproduction technology for patients with congenital bilateral absence of vas deferens.
Assisting PNA transport through cystic fibrosis human airway epithelia with biodegradable hybrid lipid-polymer nanoparticles.
Association Analyses of Genetic Polymorphisms of GSTM1, GSTT1, NQO1, NAT2, LPL, PRSS1, PSTI, and CFTR With Chronic Alcoholic Pancreatitis in Japan.
Association between Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations and Susceptibility for Childhood Asthma in Korea.
Association between F508 deletion in CFTR and chronic pancreatitis risk.
Association between serum oncofetal antigens CA 19-9 and CA 125 and clinical status in patients with cystic fibrosis.
Association of CFTR gene mutation with bronchial asthma and its severity in Indian children: a case-control study.
Association of CFTR gene mutation with bronchial asthma.
Association of CFTR gene polymorphisms with papillary thyroid cancer.
Association of Common Haplotypes of Surfactant Protein A1 and A2 (SFTPA1 and SFTPA2) Genes with Severity of Lung Disease in Cystic Fibrosis.
Association of cystic fibrosis genetic modifiers with congenital bilateral absence of the vas deferens.
Association of cystic fibrosis transmembrane conductance regulator (CFTR) mutation/variant/haplotype and tumor necrosis factor (TNF) promoter polymorphism in hyperlipidemic pancreatitis.
Association of cystic fibrosis transmembrane conductance regulator and protein phosphatase 2C.
Association of cystic fibrosis transmembrane conductance regulator gene variants with acute lung injury in African American children with pneumonia.
Association of cystic fibrosis transmembrane conductance regulator with epithelial sodium channel subunits carrying Liddle's syndrome mutations.
Association of cystic fibrosis with abnormalities in fatty acid metabolism.
Association of domains within the cystic fibrosis transmembrane conductance regulator.
Association of IVS6A GATT polymorphism of CFTR gene with cystic fibrosis: first study in CF and normal Tunisian population.
Association of M470V polymorphism of CFTR gene with variability of clinical expression of asthma: Case-report study.
Association of pancreas divisum and recurrent acute pancreatitis with the IVS8-5T-12TG allele of the CFTR gene and CFTR dysfunction.
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.
Association of S549N and IVS8-5T Splice Variants with Bronchial Asthma and Its Severity in Indian Children.
Association of the CFTR gene with asthma and airway mucus hypersecretion.
Association of the cystic fibrosis transmembrane regulator with CAL: structural features and molecular dynamics.
Association of TNF-? Gene Variants With Clinical Manifestation of Cystic Fibrosis Patients of Iranian Azeri Turkish Ethnicity.
Associations between peak oxygen uptake, lung function, and bronchiectasis in children with cystic fibrosis in the era of CFTR modulators.
Asthma and COPD in cystic fibrosis intron-8 5T carriers. A population-based study.
Asthma and pulmonary function abnormalities in heterozygotes for cystic fibrosis transmembrane regulator gene mutations.
Asymmetric 4-Aryl-1,4-dihydropyridines Potentiate Mutant Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Asymmetric structure of the cystic fibrosis transmembrane conductance regulator chloride channel pore suggested by mutagenesis of the twelfth transmembrane region.
Asymptomatic cystic fibrosis diagnosed in an adult evaluated for hematuria.
Ataluren (PTC124) Induces CFTR Protein Expression and Activity in Children with Nonsense Mutation Cystic Fibrosis.
Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis.
Ataluren/ivacaftor combination therapy: Two N-of-1 trials in cystic fibrosis patients with nonsense mutations.
Atomic model of human cystic fibrosis transmembrane conductance regulator: membrane-spanning domains and coupling interfaces.
Atomic Structure of the Cystic Fibrosis Transmembrane Conductance Regulator.
ATP alters current fluctuations of cystic fibrosis transmembrane conductance regulator: evidence for a three-state activation mechanism.
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.
ATP binding cassette modulators control abscisic acid-regulated slow anion channels in guard cells
ATP depletion induces a loss of respiratory epithelium functional integrity and down-regulates CFTR (cystic fibrosis transmembrane conductance regulator) expression.
ATP hydrolysis by a CFTR domain: pharmacology and effects of G551D mutation.
ATP hydrolysis-dependent asymmetry of the conformation of CFTR channel pore.
ATP release triggered by activation of the Ca2+-activated K+ channel in human airway Calu-3 cells.
ATP secretion from nerve trunks and Schwann cells mediated by glutamate.
ATP-binding cassette (ABC) transporters in normal and pathological lung.
ATP-dependent activation of K(Ca) and ROMK-type K(ATP) channels in human submandibular gland ductal cells.
ATP-Dependent Signaling in Simulations of a Revised Model of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
ATP-dependent transport systems in bacteria and humans: relevance to cystic fibrosis and multidrug resistance.
ATP-independent CFTR channel gating and allosteric modulation by phosphorylation.
ATP-induced Cl- secretion with suppressed Na+ absorption in rabbit tracheal epithelium.
ATPase activity of the cystic fibrosis transmembrane conductance regulator.
ATPase assay of purified, reconstituted CFTR protein.
Atrial natriuretic peptide modulates cystic fibrosis transmembrane conductance regulator chloride channel expression in rat proximal colon and human intestinal epithelial cells.
Attenuation of Phosphorylation-dependent Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Disease-causing Mutations at the Transmission Interface.
Atypical 5' splice sites cause CFTR exon 9 to be vulnerable to skipping.
Atypical sinusitis in adults must lead to looking for cystic fibrosis and primary ciliary dyskinesia.
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.
Auto-obliteration of maxillary sinuses through osteoneogenesis in children with cystic fibrosis: A possible new way to reduce morbidity.
Automated analysis of multiplex microsatellites.
Autophagy and inflammation in chronic respiratory disease.
Autophagy Augmentation to Alleviate Immune Response Dysfunction, and Resolve Respiratory and COVID-19 Exacerbations.
Autosomal dominant polycystic kidney disease coexisting with cystic fibrosis.
Azaspiracids Increase Mitochondrial Dehydrogenases Activity in Hepatocytes: Involvement of Potassium and Chloride Ions.
Azithromycin and tezacaftor/ivacaftor is associated with first-degree heart block in an adult with cystic fibrosis.
Azithromycin fails to reduce inflammation in cystic fibrosis airway epithelial cells.
Bacterial host interactions in cystic fibrosis.
Bacterial overgrowth in the cystic fibrosis transmembrane conductance regulator null mouse small intestine.
Bacterial periplasmic permeases as model systems for multidrug resistance (MDR) and the cystic fibrosis transmembrane conductance regulator (CFTR).
Bacterial periplasmic permeases as model systems for the superfamily of traffic ATPases, including the multidrug resistance protein and the cystic fibrosis transmembrane conductance regulator.
Bacterial Sphingomyelinase is a State-Dependent Inhibitor of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR).
Bacterial transfer of large functional genomic DNA into human cells.
Bafilomycin A1-sensitive pathway is required for the maturation of cystic fibrosis transmembrane conductance regulator.
BAG-1 stabilizes mutant F508del-CFTR in a ubiquitin-like-domain-dependent manner.
BAG-2 acts as an inhibitor of the chaperone-associated ubiquitin ligase CHIP.
Basal chloride currents in murine airway epithelial cells: modulation by CFTR.
Basal expression of the cystic fibrosis transmembrane conductance regulator gene is dependent on protein kinase A activity.
Basal nucleotide levels, release, and metabolism in normal and cystic fibrosis airways.
Basal Serum Cortisol and Testosterone/Cortisol Ratio Are Related to Rate of Na+ Lost During Exercise in Elite Soccer Players.
Base treatment corrects defects due to misfolding of mutant cystic fibrosis transmembrane conductance regulator.
Basic protocol for transepithelial nasal potential difference measurements.
Basolateral chloride current in human airway epithelia.
Basolateral chloride transporters in autosomal dominant polycystic kidney disease.
Bcl-2 suppresses sarcoplasmic/endoplasmic reticulum Ca2+-ATPase expression in cystic fibrosis airways: role in oxidant-mediated cell death.
Benefit-Risk Assessment of Plecanatide in the Treatment of Chronic Idiopathic Constipation.
Benign and Deleterious Cystic Fibrosis Transmembrane Conductance Regulator Mutations Identified by Sequencing in Positive Cystic Fibrosis Newborn Screen Children from California.
Benign outcome among positive cystic fibrosis newborn screen children with non-CF-causing variants.
Benzo(c)quinolizinium drugs inhibit degradation of Delta F508-CFTR cytoplasmic domain.
Benzoflavone activators of the cystic fibrosis transmembrane conductance regulator: towards a pharmacophore model for the nucleotide-binding domain.
Benzopyrimido-pyrrolo-oxazine-dione (R)-BPO-27 Inhibits CFTR Chloride Channel Gating by Competition with ATP.
Benzopyrimido-pyrrolo-oxazine-dione CFTR inhibitor (R)-BPO-27 for antisecretory therapy of diarrheas caused by bacterial enterotoxins.
Beta-2-adrenergic receptor polymorphisms in cystic fibrosis.
Beta-adrenergic receptors couple to CFTR chloride channels of intercalated mitochondria-rich cells in the heterocellular toad skin epithelium.
Beta-lactam allergy in adults with cystic fibrosis.
beta3-adrenoceptor control the cystic fibrosis transmembrane conductance regulator through a cAMP/protein kinase A-independent pathway.
Beyond cystic fibrosis transmembrane conductance regulator (CFTR) single channel kinetics: implications for therapeutic intervention.
Beyond cystic fibrosis transmembrane conductance regulator therapy: a perspective on gene therapy and small molecule treatment for cystic fibrosis.
Bicarbonate and chloride secretion in Calu-3 human airway epithelial cells.
Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype.
Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator.
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.
Bicarbonate secretion in the murine gallbladder--lessons for the treatment of cystic fibrosis.
Bicarbonate secretion of mouse cholangiocytes involves Na(+)-HCO(3)(-) cotransport in addition to Na(+)-independent Cl(-)/HCO(3)(-) exchange.
Bicarbonate-rich choleresis induced by secretin in normal rat is taurocholate-dependent and involves AE2 anion exchanger.
Bicarbonate-rich fluid secretion predicted by a computational model of guinea-pig pancreatic duct epithelium.
Big data and stratified medicine: what does it mean for children?
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.
Bile acids stimulate chloride secretion through CFTR and calcium-activated Cl- channels in Calu-3 airway epithelial cells.
Bile-derived organoids from patients with primary sclerosing cholangitis recapitulate their inflammatory immune profile.
Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis.
Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator.
Binding of serum response factor to cystic fibrosis transmembrane conductance regulator CArG-like elements, as a new potential CFTR transcriptional regulation pathway.
Binding screen for cystic fibrosis transmembrane conductance regulator correctors finds new chemical matter and yields insights into cystic fibrosis therapeutic strategy.
Binding site of activators of the cystic fibrosis transmembrane conductance regulator in the nucleotide binding domains.
Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity.
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.
Biochemical and biophysical approaches to probe CFTR structure.
Biochemical and biophysical identification of cystic fibrosis transmembrane conductance regulator chloride channels as components of endocytic clathrin-coated vesicles.
Biochemical basis of the interaction between cystic fibrosis transmembrane conductance regulator (CFTR) and immunoglobulin-like repeats of filamin.
Biochemical characterization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis epithelial cells.
Biochemical evidence for ATPase activity in CFTR-enriched apical membrane vesicles from tracheal epithelium.
Biochemical implications of sequence comparisons of the cystic fibrosis transmembrane conductance regulator.
Biochemical methods to assess CFTR expression and membrane localization.
Biofilm-dependent airway infections: A role for ambroxol?
Biogenesis of CFTR and other polytopic membrane proteins: new roles for the ribosome-translocon complex.
Biological and structural basis for Aha1 regulation of Hsp90 ATPase activity in maintaining proteostasis in the human disease cystic fibrosis.
Biological Characterization of F508delCFTR Protein Processing by the CFTR Corrector ABBV-2222/GLPG2222.
Biomarkers for cystic fibrosis drug development.
Biomarkers in Paediatric Cystic Fibrosis Lung Disease.
Biophysical characterisation of calumenin as a charged F508del-CFTR folding modulator.
Biopolymer synthesis on polypropylene supports: oligonucleotide arrays.
Biosynthesis and degradation of CFTR.
Biosynthesis of cystic fibrosis transmembrane conductance regulator.
Biosynthetic and growth abnormalities are associated with high-level expression of CFTR in heterologous cells.
Birth after intracytoplasmic injection of epididymal sperm from a man with congenital bilateral absence of the vas deferens who had a robertsonian translocation.
Birth of a normal girl after in vitro fertilization and preimplantation diagnostic testing for cystic fibrosis.
Bithiazole Correctors Rescue CFTR Mutants by Two Different Mechanisms.
Block by fluoxetine of volume-regulated anion channels.
Block by MOPS reveals a conformation change in the CFTR pore produced by ATP hydrolysis.
Block of CFTR-dependent chloride currents by inhibitors of multidrug resistance-associated proteins.
Blood concentrations of pancreatitis associated protein in neonates: relevance to neonatal screening for cystic fibrosis.
Blood immunoreactive trypsinogen concentrations are genetically determined in healthy and cystic fibrosis newborns.
Blue native/SDS-PAGE analysis reveals reduced expression of the mClCA3 protein in cystic fibrosis knock-out mice.
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.
Bone Cells Differentiation: How CFTR Mutations May Rule the Game of Stem Cells Commitment?
Bone disease in cystic fibrosis: new pathogenic insights opening novel therapies.
Borderline sweat test: Utility and limits of genetic analysis for the diagnosis of cystic fibrosis.
Both CFTR and outwardly rectifying chloride channels contribute to cAMP-stimulated whole cell chloride currents.
Both the wild type and a functional isoform of CFTR are expressed in kidney.
Bovine AAV transcytosis inhibition by tannic acid results in functional expression of CFTR in vitro and altered biodistribution in vivo.
Bowel ultrasound imaging in patients with cystic fibrosis: Relationship with clinical symptoms and CFTR genotype.
Branchial ionocyte organization and ion-transport protein expression in juvenile alewives acclimated to freshwater or seawater.
Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors.
Bringing new treatments to the bedside in 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.
Buccal cell DNA mutation analysis for diagnosis of cystic fibrosis in newborns and infants inaccessible to sweat chloride measurement.
Building an understanding of cystic fibrosis on the foundation of ABC transporter structures.
Bumetanide blocks CFTR GCl in the native sweat duct.
Burkholderia cenocepacia-induced delay of acidification and phagolysosomal fusion in cystic fibrosis transmembrane conductance regulator (CFTR)-defective macrophages.
Buserelin alleviates chloride transport defect in human cystic fibrosis nasal epithelial cells.
Butyrate increases apical membrane CFTR but reduces chloride secretion in MDCK cells.
c-Cbl facilitates endocytosis and lysosomal degradation of cystic fibrosis transmembrane conductance regulator in human airway epithelial cells.
C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis. A novel class of mutation.
C-type natriuretic peptide and its receptor are downregulated in pulmonary epithelium following birth.
C-type natriuretic peptide increases chloride permeability in normal and cystic fibrosis airway cells.
c.753_754delAG, a novel CFTR mutation found in a Chinese patient with cystic fibrosis: A case report and review of the literature.
CA/GT microsatellite alleles within the cystic fibrosis transmembrane conductance regulator (CFTR) gene are not generated by unequal crossingover.
Ca2+ and cAMP-activated Cl- conductances mediate Cl- secretion in a mouse renal inner medullary collecting duct cell line.
Calcitonin Receptor-Mediated CFTR Activation in Human Intestinal Epithelial Cells.
Calcium is not involved in the cAMP-mediated stimulation of Cl- conductance in the apical membrane of Necturus gallbladder epithelium.
Calcium Modulated Chloride Pathways Contribute to Chloride Flux in Murine CF-Affected Macrophages.
Calcium-activated chloride conductance is not increased in pancreatic duct cells of CF mice.
Calcium-activated chloride currents and non-selective cation channels in a novel cystic fibrosis-derived human pancreatic duct cell line.
Calcium-pump inhibitors induce functional surface expression of Delta F508-CFTR protein in cystic fibrosis epithelial cells.
Calculating expected lung deposition of aerosolized administration of AAV vector in human clinical studies.
Calculating posterior cystic fibrosis risk with echogenic bowel and one characterized cystic fibrosis mutation: avoiding pitfalls in the risk calculations.
CALEB/NGC interacts with the Golgi-associated protein PIST.
Calnexin Delta 185-520 partially reverses the misprocessing of the Delta F508 cystic fibrosis transmembrane conductance regulator.
Calpain inhibition promotes the rescue of F(508)del-CFTR in PBMC from cystic fibrosis patients.
Calreticulin negatively regulates the cell surface expression of cystic fibrosis transmembrane conductance regulator.
Calu-3: a human airway epithelial cell line that shows cAMP-dependent Cl- secretion.
cAMP activates an ATP-permeable pathway in neonatal rat cardiac myocytes.
cAMP activation of chloride and fluid secretion across the rabbit alveolar epithelium.
cAMP and genistein stimulate HCO3- conductance through CFTR in human airway epithelia.
cAMP inhibition of murine intestinal Na/H exchange requires CFTR-mediated cell shrinkage of villus epithelium.
cAMP regulates cell proliferation and cyst formation in autosomal polycystic kidney disease cells.
cAMP regulation of Cl(-) and HCO(-)(3) secretion across rat fetal distal lung epithelial cells.
cAMP stimulates CFTR-like Cl- channels and inhibits amiloride-sensitive Na+ channels in mouse CCD cells.
cAMP Stimulates SLC26A3 Activity in Human Colon by a CFTR-Dependent Mechanism That Does Not Require CFTR Activity.
cAMP stimulation of CFTR-expressing Xenopus oocytes activates a chromanol-inhibitable K+ conductance.
cAMP stimulation of HCO3- secretion across airway epithelia.
cAMP- and Ca2+-independent activation of cystic fibrosis transmembrane conductance regulator channels by phenylimidazothiazole drugs.
cAMP- but not Ca(2+)-regulated Cl- conductance in the oviduct is defective in mouse model of cystic fibrosis.
cAMP- but not Ca(2+)-regulated Cl- conductance is lacking in cystic fibrosis mice epididymides and seminal vesicles.
cAMP-activated Cl channels in CFTR-transfected cystic fibrosis pancreatic epithelial cells.
cAMP-activated Cl- channels in primary cultures of spiny dogfish (Squalus acanthias) rectal gland.
cAMP-dependent activation of CFTR inhibits the epithelial sodium channel (ENaC) without affecting its surface expression.
cAMP-dependent activation of ion conductances in bronchial epithelial cells.
cAMP-dependent chloride secretion mediates tubule enlargement and cyst formation by cultured mammalian collecting duct cells.
cAMP-dependent exocytosis and vesicle traffic regulate CFTR and fluid transport in rat jejunum in vivo.
cAMP-dependent protein kinase-mediated phosphorylation of cystic fibrosis transmembrane conductance regulator residue Ser-753 and its role in channel activation.
cAMP-independent activation of CFTR Cl channels by the tyrosine kinase inhibitor genistein.
cAMP-independent phosphorylation activation of CFTR by G proteins in native human sweat duct.
cAMP-independent regulation of CFTR by the actin cytoskeleton.
cAMP-induced chloride transport in NCL-SG3 sweat gland cells.
cAMP-inducible chloride conductance in mouse fibroblast lines stably expressing the human cystic fibrosis transmembrane conductance regulator.
cAMP-regulated trafficking of epitope-tagged CFTR.
cAMP-regulated whole cell chloride currents in pancreatic duct cells.
cAMP-stimulated ion currents in Xenopus oocytes expressing CFTR cRNA.
cAMP/protein kinase A activates cystic fibrosis transmembrane conductance regulator for ATP release from rat skeletal muscle during low pH or contractions.
Campylobacter jejuni infection suppressed Cl? secretion induced by CFTR activation in T-84 cells.
Can a polymorphism in the thalassemia gene and a heterozygote CFTR mutation cause acute pancreatitis?
Can Cystic Fibrosis Patients Finally Catch a Breath With Lumacaftor/Ivacaftor?
Can intervention in inositol phosphate signalling pathways improve therapy for cystic fibrosis?
Can Tangier disease cause male infertility? A case report and an overview on genetic causes of male infertility and hormonal axis involved.
Can Targeting Iron Help in Combating Chronic Pseudomonas Infection? A Systematic Review.
Capacitance measurements reveal different pathways for the activation of CFTR.
Capacitation-associated alkalization in human sperm is differentially controlled at the subcellular level.
Capsaicin potentiates wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride-channel currents.
Capsaicin-Loaded Chitosan Nanocapsules for wtCFTR-mRNA Delivery to a Cystic Fibrosis Cell Line.
Capsaicinoids regulate airway anion transporters through Rho kinase- and cAMP-dependent mechanisms.
Carbachol-induced MUC17 endocytosis is concomitant with NHE3 internalization and CFTR membrane recruitment in enterocytes.
Carbamazepine-induced sperm disorders can be associated with the altered expressions of testicular KCNJ11/miR-let-7a and spermatozoal CFTR/miR-27a.
Carbon monoxide-releasing molecules inhibit the cystic fibrosis transmembrane conductance regulator Cl- channel.
Carbon nanofiber-based multiplexed immunosensor for the detection of survival motor neuron 1, cystic fibrosis transmembrane conductance regulator and Duchenne Muscular Dystrophy proteins.
Cardiac expression of the cystic fibrosis transmembrane conductance regulator involves novel exon 1 usage to produce a unique amino-terminal protein.
Cardiac ion channel current modulation by the CFTR inhibitor GlyH-101.
Cardiomyocytes with disrupted CFTR function require CaMKII and Ca2+-activated Cl- channel activity to maintain contraction rate.
Cardiovascular complications in cystic fibrosis: A review of the literature.
Cardiovascular System Involvement in Cystic Fibrosis.
Caring for Adolescents with Cystic Fibrosis, in Portugal: The Nurse's Role.
Carrier detection and prenatal diagnosis of cystic fibrosis using an intragenic TA-repeat polymorphism.
Carvedilol binding to ?2-adrenergic receptors inhibits CFTR-dependent anion secretion in airway epithelial cells.
Catalyst-like modulation of transition states for CFTR channel opening and closing: new stimulation strategy exploits nonequilibrium gating.
Catamenial haemoptysis in females with cystic fibrosis: a case series with review of management strategies.
Catch me if you can! Novel aspects of cadmium transport in mammalian cells.
Categories of deltaF508 homozygous cystic fibrosis twin and sibling pairs with distinct phenotypic characteristics.
Cationic lipid formulations for intracellular gene delivery of cystic fibrosis transmembrane conductance regulator to airway epithelia.
Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis: a double-blind placebo-controlled trial.
Cationic lipid:pDNA complexes for the treatment of cystic fibrosis.
Cationic lipids for reporter gene and CFTR transfer to rat pulmonary epithelium.
Cefoperazone sodium liposomal formulation to mitigate P. aeruginosa biofilm in Cystic fibrosis infection: A QbD approach.
Ceftolozane/tazobactam for pulmonary exacerbation in a 63-year-old cystic fibrosis patient with renal insufficiency and an elevated MIC to Pseudomonas aeruginosa.
CELF proteins regulate CFTR pre-mRNA splicing: essential role of the divergent domain of ETR-3.
Celiac Disease in Patients With Cystic Fibrosis on Ivacaftor: A Case Series.
Cell culture models demonstrate that CFTR dysfunction leads to defective fatty acid composition and metabolism.
Cell engineering method using fluorogenic oligonucleotide signaling probes and flow cytometry.
Cell surface dynamics of CFTR: the ins and outs.
Cell surface expression and turnover of the alpha-subunit of the epithelial sodium channel.
Cell surface labeling of CFTR in T84 cells.
Cell volume response to hyposmotic shock and elevated cAMP in bovine trabecular meshwork cells.
Cell Volume-Activated and Volume-Correlated Anion Channels in Mammalian Cells: Their Biophysical, Molecular, and Pharmacological Properties.
Cell-based assay for high-throughput quantitative screening of CFTR chloride transport agonists.
Cell-Based Therapeutic Approaches for Cystic Fibrosis.
Cell-Selective Regulation of CFTR Gene Expression: Relevance to Gene Editing Therapeutics.
Cell-type-specific long-range looping interactions identify distant regulatory elements of the CFTR gene.
Cellular and viral splicing factors can modify the splicing pattern of CFTR transcripts carrying splicing mutations.
Cellular ATP release by the cystic fibrosis transmembrane conductance regulator.
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.
Cellular differentiation regulates expression of Cl- transport and cystic fibrosis transmembrane conductance regulator mRNA in human intestinal cells.
Cellular distribution and function of ion channels involved in transport processes in rat tracheal epithelium.
Cellular effects of guanylin and uroguanylin.
Cellular localization and activity of Ad-delivered GFP-CFTR in airway epithelial and tracheal cells.
Cellular localization of cystic fibrosis transmembrane regulator protein in piglet and mouse intestine.
Cellular localization of the cystic fibrosis transmembrane conductance regulator in mouse intestinal tract.
Cellular mechanism for potentiation of Ca2+-mediated Cl- secretion by the flavonoid baicalein in intestinal epithelia.
Cellular mechanism underlying oxytocin-stimulated Cl- secretion in rat cauda epididymal epithelium.
Cellular mechanisms underlying carbon monoxide stimulated anion secretion in rat epididymal epithelium.
Cellular Model of TAR DNA-binding Protein 43 (TDP-43) Aggregation Based on Its C-terminal Gln/Asn-rich Region.
Cellular response to cigarette smoke and oxidants: adapting to survive.
Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis.
Ceramide in cystic fibrosis.
Ceramide in Pseudomonas aeruginosa infections and cystic fibrosis.
Certain background factors exhibit an association with an increased risk for pancreatic calcification among Japanese male alcoholics.
CF gene and cystic fibrosis transmembrane conductance regulator expression in autosomal dominant polycystic kidney disease.
CF2603/4delT, a new frameshift mutation in exon 13 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
CFTR 5T variant has a low penetrance in females that is partially attributable to its haplotype.
CFTR activation in human bronchial epithelial cells by novel benzoflavone and benzimidazolone compounds.
CFTR activation suppresses glioblastoma cell proliferation, migration and invasion.
CFTR activation: additive effects of stimulatory and inhibitory phosphorylation sites in the R domain.
CFTR activator increases intestinal fluid secretion and normalizes stool output in a mouse model of constipation.
CFTR activity and mitochondrial function.
CFTR and Anoctamin 1 (ANO1) contribute to cAMP amplified exocytosis and insulin secretion in human and murine pancreatic beta-cells.
CFTR and bicarbonate secretion by [correction of to] epithelial cells.
CFTR and calcium-activated chloride channels in primary cultures of human airway gland cells of serous or mucous phenotype.
CFTR and calcium-activated chloride currents in pancreatic duct cells of a transgenic CF mouse.
CFTR and defective endocytosis: new insights in the renal phenotype of cystic fibrosis.
Cftr and ENaC ion channels mediate NaCl absorption in the mouse submandibular gland.
CFTR and its key role in in vivo resting and luminal acid-induced duodenal HCO3- secretion.
CFTR and lysozyme secretion in human airway epithelial cells.
CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship.
CFTR and sphingolipids mediate hypoxic pulmonary vasoconstriction.
CFTR and Tight Junctions in Cultured Bronchial Epithelial Cells.
CFTR and TMEM16A are separate but functionally related Cl- channels.
CFTR and TNR-CFTR expression and function in the kidney.
CFTR and Wnt/beta-catenin signaling in lung development.
CFTR anion channel modulates expression of human transmembrane mucin MUC3 through the PDZ protein GOPC.
CFTR antisense phosphorothioate oligodeoxynucleotides (S-ODns) induce tracheo-bronchial mucin (TBM) mRNA expression in human airway mucosa.
CFTR as a cAMP-dependent regulator of sodium channels.
CFTR channel in oocytes from Xenopus laevis and its regulation by xShroom1 protein.
CFTR channel insertion to the apical surface in rat duodenal villus epithelial cells is upregulated by VIP in vivo.
CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains.
CFTR channels expressed in CHO cells do not have detectable ATP conductance.
CFTR chloride channel activation by genistein: the role of serine/threonine protein phosphatases.
CFTR chloride channel activity modulates the mitochondrial morphology in cultured epithelial cells.
CFTR chloride channel drug discovery--inhibitors as antidiarrheals and activators for therapy of cystic fibrosis.
CFTR chloride channel in the apical compartments: spatiotemporal coupling to its interacting partners.
CFTR chloride channel is a molecular target of the natural cancer preventive agent resveratrol.
CFTR chloride channels are regulated by a SNAP-23/syntaxin 1A complex.
CFTR chloride channels in human and simian heart.
CFTR Cl- channel and CFTR-associated ATP channel: distinct pores regulated by common gates.
CFTR Cl- channel function in native human colon correlates with the genotype and phenotype in 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.
CFTR constrains the differentiation from mouse embryonic stem cells to intestine lineage cells.
Cftr controls lumen expansion and function of Kupffer's vesicle in zebrafish.
CFTR Cooperative Cis-Regulatory Elements in Intestinal Cells.
CFTR corrector C17 is effective in muscular dystrophy, in vivo proof of concept in LGMDR3.
CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells.
CFTR Deficiency Affects Glucose Homeostasis via Regulating GLUT4 Plasma Membrane Transportation.
CFTR Deletion Confers Mitochondrial Dysfunction and Disrupts Lipid Homeostasis in Intestinal Epithelial Cells.
CFTR deltaF508 carrier status, risk of breast cancer before the age of 40 and histological grading in a population-based case-control study.
CFTR DeltaF508 mutation has minimal effect on the gene expression profile of differentiated human airway epithelia.
CFTR Depletion Confers Hypersusceptibility to Mycobacterium fortuitum in a Zebrafish Model.
CFTR depletion results in changes in fatty acid composition and promotes lipogenesis in intestinal Caco 2/15 cells.
CFTR directly mediates nucleotide-regulated glutathione flux.
CFTR disruption impairs cAMP-dependent Cl(-) secretion in primary cultures of mouse cortical collecting ducts.
CFTR dysfunction in cystic fibrosis and chronic obstructive pulmonary disease.
CFTR dysfunction predisposes to fibrotic liver disease in a murine model.
CFTR Expression Analysis for Subtyping of Human Pancreatic Cancer Organoids.
CFTR expression and chloride secretion in polarized immortal human bronchial epithelial cells.
CFTR expression and mucin secretion in cultured mouse gallbladder epithelial cells.
CFTR expression and organ damage in 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.
CFTR expression in cortical collecting duct cells.
CFTR expression regulation by the unfolded protein response.
CFTR fails to inhibit the epithelial sodium channel ENaC expressed in Xenopus laevis oocytes.
CFTR function and clinical response to modulators parallel nasal epithelial organoid swelling.
CFTR function and prospects for therapy.
CFTR functions as a bicarbonate channel in pancreatic duct cells.
CFTR Functions as a Tumor Suppressor and Is Regulated by DNA Methylation in Colorectal Cancer.
CFTR gating II: Effects of nucleotide binding on the stability of open states.
CFTR gene analysis in cystic fibrosis patients: detection of 91% of molecular defects and identification of the novel mutation D979V.
CFTR gene mutation spectrum among 735 Iranian patients with cystic fibrosis: A comprehensive systematic review.
CFTR gene mutations and asthma in the Norwegian Environment and Childhood Asthma study.
CFTR gene mutations and male infertility.
CFTR gene mutations and polymorphism are associated with non-obstructive azoospermia: From case-control study.
CFTR gene mutations in adults with disseminated bronchiectasis.
CFTR gene mutations in pancreatitis: Frequency and clinical manifestations in an Austrian patient cohort.
CFTR gene mutations in sarcoidosis.
CFTR gene mutations--including three novel nucleotide substitutions--and haplotype background in patients with asthma, disseminated bronchiectasis and chronic obstructive pulmonary disease.
CFTR gene transfer corrects defective glycoconjugate secretion in human CF epithelial tracheal cells.
CFTR gene transfer to lung epithelium--on the trail of a target cell.
CFTR gene: molecular analysis in patients from South Brazil.
CFTR genotype and clinical outcomes of adult patients carried as cystic fibrosis disease.
CFTR Genotype and Maximal Exercise Capacity in Cystic Fibrosis: A Cross-sectional Study.
CFTR haplotype analysis reveals genetic heterogeneity in the etiology of congenital bilateral aplasia of the vas deferens.
CFTR haplotype distribution in the Brazilian Western Amazonian region.
CFTR haplotypes in northern Iranian population.
CFTR haplotypic variability for normal and mutant genes in cystic fibrosis families from southern France.
CFTR Heterozygotes Are at Increased Risk of Respiratory Infections: A Population-Based Study.
CFTR illegitimate transcription in lymphoid cells: quantification and applications to the investigation of pathological transcripts.
CFTR impairment upregulates c-Src activity through IL-1? autocrine signaling.
CFTR in a lipid raft-TNFR1 complex modulates gap junctional intercellular communication and IL-8 secretion.
CFTR in Calu-3 human airway cells: channel properties and role in cAMP-activated Cl- conductance.
CFTR Inactivation by Lentiviral Vector-mediated RNA Interference and CRISPR-Cas9 Genome Editing in Human Airway Epithelial Cells.
CFTR induces extracellular acid sensing in Xenopus oocytes which activates endogenous Ca(2+)-activated Cl (-) conductance.
CFTR induces the expression of DRA along with Cl(-)/HCO(3)(-) exchange activity in tracheal epithelial cells.
CFTR Influences Beta Cell Function and Insulin Secretion Through Non-Cell Autonomous Exocrine-Derived Factors.
CFTR inhibition augments NHE3 activity during luminal high CO2 exposure in rat duodenal mucosa.
CFTR Inhibitors for Treating Diarrheal Disease.
CFTR Inhibitors.
CFTR inhibits the invasion and growth of esophageal cancer cells by inhibiting the expression of NF-?B.
CFTR interacts with Hsp90 and regulates the phosphorylation of AKT and ERK1/2 in colorectal cancer cells.
CFTR intron 1 increases luciferase expression driven by CFTR 5'-flanking DNA in a yeast artificial chromosome.
CFTR involvement in chloride, bicarbonate, and liquid secretion by airway submucosal glands.
CFTR is a conductance regulator as well as a chloride channel.
CFTR is a mechanosensitive anion channel: a real stretch?
CFTR is a pattern recognition molecule that extracts Pseudomonas aeruginosa LPS from the outer membrane into epithelial cells and activates NF-kappa B translocation.
CFTR is a potential marker for nasopharyngeal carcinoma prognosis and metastasis.
CFTR is activated through stimulation of purinergic P2Y(2) receptors.
CFTR is essential for sperm fertilizing capacity and is correlated with sperm quality in humans.
CFTR is functionally active in GnRH-expressing GT1-7 hypothalamic neurons.
CFTR is involved in membrane endocytosis but not in fluid-phase and receptor-mediated endocytosis in human respiratory epithelial cells.
CFTR is involved in the regulation of glucagon secretion in human and rodent alpha cells.
CFTR is required for cAMP inhibition of intestinal Na+ absorption in a cystic fibrosis mouse model.
CFTR is required for cellular entry and internalization of Chlamydia trachomatis.
CFTR is required for maximal transepithelial liquid transport in pig alveolar epithelia.
CFTR is required for PKA-regulated ATP sensitivity of Kir1.1 potassium channels in mouse kidney.
CFTR is required for the migration of primordial germ cells during zebrafish early embryogenesis.
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.
CFTR IVS8 Poly-T Variation Affects Severity of Acute Pancreatitis in Women.
CFTR Knockdown induces proinflammatory changes in intestinal epithelial cells.
CFTR KNOCKDOWN STIMULATES LIPID SYNTHESIS AND TRANSPORT IN INTESTINAL CACO-2/15 CELLS.
CFTR localization in native airway cells and cell lines expressing wild-type or F508del-CFTR by a panel of different antibodies.
CFTR may modulate AQP9 functionality in preeclamptic placentas.
CFTR may play a role in regulated secretion by lymphocytes: a new hypothesis for the pathophysiology of cystic fibrosis.
CFTR mediates cAMP- and Ca2+-activated duodenal epithelial HCO3- secretion.
CFTR mediates electrogenic chloride secretion in mouse inner medullary collecting duct (mIMCD-K2) cells.
CFTR mediates noradrenaline-induced ATP efflux from DRG neurons.
CFTR modulates programmed cell death by decreasing intracellular pH in Chinese hamster lung fibroblasts.
CFTR modulates RPS27 gene expression using chloride anion as signaling effector.
Cftr Modulates Wnt/?-Catenin Signaling and Stem Cell Proliferation in Murine Intestine.
CFTR modulator drug desensitization: Preserving the hope of long term improvement.
CFTR Modulator Therapies in Pediatric Cystic Fibrosis: Focus on Ivacaftor.
CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis.
CFTR modulator theratyping: Current status, gaps and future directions.
CFTR modulator use in post lung transplant recipients.
CFTR Modulators: Impact on Fertility, Pregnancy, and Lactation in Women with Cystic Fibrosis.
CFTR mRNA expression is regulated by an upstream open reading frame and RNA secondary structure in its 5' untranslated region.
CFTR Mutation Analysis of a Caucasian Father with Congenital Bilateral Absence of Vas Deferens, a Taiwanese Mother, and Twins Resulting from ICSI Procedure.
CFTR mutation enhances Dishevelled degradation and results in impairment of Wnt-dependent hematopoiesis.
CFTR mutations and host susceptibility to Pseudomonas aeruginosa lung infection.
CFTR mutations and polymorphisms in male infertility.
CFTR mutations impart elevated immune reactivity in a murine model of cystic fibrosis related diabetes.
CFTR mutations spectrum and the efficiency of molecular diagnostics in Polish cystic fibrosis patients.
CFTR negatively regulates cyclooxygenase-2-PGE(2) positive feedback loop in inflammation.
CFTR null mutation altered cAMP-sensitive and swelling-activated Cl- currents in primary cultures of mouse nephron.
CFTR pharmacology and its role in intestinal fluid secretion.
CFTR polymorphisms of healthy individuals in two Chinese cities--Changchun and Nanjing.
CFTR POTENTIATOR VX-770 (IVACAFTOR) OPENS THE DEFECTIVE CHANNEL GATE OF MUTANT CFTR IN A PHOSPHORYLATION-DEPENDENT BUT ATP-INDEPENDENT MANNER.
CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR.
CFTR prevents neuronal apoptosis following cerebral ischemia reperfusion via regulating mitochondrial oxidative stress.
CFTR processing, trafficking and interactions.
CFTR promotes malignant glioma development via up-regulation of Akt/Bcl2-mediated anti-apoptosis pathway.
CFTR protects against vascular inflammation and atherogenesis in apolipoprotein E-deficient mice.
CFTR regulates acute inflammatory responses in macrophages.
CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP.
CFTR regulates phagosome acidification in macrophages and alters bactericidal activity.
CFTR Regulates the Proliferation, Migration and Invasion of Cervical Cancer Cells by Inhibiting the NF-?B Signalling Pathway.
CFTR regulation by phosphorylation.
CFTR regulation in human airway epithelial cells requires integrity of the actin cytoskeleton and compartmentalized cAMP and PKA activity.
CFTR regulation of aquaporin-mediated water transport.
CFTR Regulation of Aquaporin-Mediated Water Transport: A Target in Male Fertility.
CFTR regulation of epithelial sodium channel.
CFTR Regulation of Intracellular pH and Ceramides is Required for Lung Endothelial Cell Apoptosis.
CFTR regulatory region interacts with NBD1 predominantly via multiple transient helices.
CFTR silencing in pancreatic ?-cells reveals a functional impact on glucose-stimulated insulin secretion and oxidative stress response.
CFTR structure, stability, function and regulation.
CFTR structure.
CFTR supports cell death through ROS-dependent activation of TMEM16F (anoctamin 6).
CFTR surface expression and chloride currents are decreased by inhibitors of N-WASP and actin polymerization.
CFTR targeted therapies: recent advances in cystic fibrosis and possibilities in other diseases of the airways.
CFTR targeting during activation of human neutrophils.
CFTR Therapeutics Normalize Cerebral Perfusion Deficits in Mouse Models of Heart Failure and Subarachnoid Hemorrhage.
CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates.
CFTR transcription defects in pancreatic sufficient cystic fibrosis patients with only one mutation in the coding region of CFTR.
CFTR transgene expression in primary DeltaF508 epithelial cell cultures from human nasal polyps following gene transfer with cationic phosphonolipids.
CFTR transmembrane segments are impaired in their conformational adaptability by a pathogenic loop mutation and dynamically stabilized by Lumacaftor.
CFTR upregulates the expression of the basolateral Na(+)-K(+)-2Cl(-) cotransporter in cultured pancreatic duct cells.
CFTR's proteolysis protection. The cystic fibrosis transmembrane conductance regulator impedes proteolytic stimulation of the epithelial Na+ channel.
CFTR, a channel with the structure of a transporter.
CFTR, bicarbonate, and the pathophysiology of cystic fibrosis.
CFTR, investigated with the two-electrode voltage-clamp technique: the importance of knowing the series resistance.
CFTR, Mucins, and Mucus Obstruction in Cystic Fibrosis.
CFTR, PRSS1 and SPINK1 mutations in the development of pancreatitis in Brazilian patients.
CFTR, SPINK1, CTRC and PRSS1 variants in chronic pancreatitis: is the role of mutated CFTR overestimated?
CFTR, SPINK1, PRSS1, and CTRC Mutations Are Not Associated With Pancreatic Cancer in German Patients.
CFTR-?-catenin interaction regulates mouse embryonic stem cell differentiation and embryonic development.
CFTR-associated ligand is a negative regulator of Mrp2 expression.
CFTR-deficiency renders mice highly susceptible to cutaneous symptoms during mite infestation.
CFTR-deficient pigs display alterations of bone microarchitecture and composition at birth.
CFTR-deficient pigs display peripheral nervous system defects at birth.
CFTR-dependent chloride efflux in cystic fibrosis mononuclear cells is increased by ivacaftor therapy.
CFTR-dependent Cl- secretion in Xenopus laevis lung epithelium.
CFTR-dependent membrane insertion is linked to stimulation of the CFTR chloride conductance.
CFTR-function and ventilation inhomogeneity in individuals with cystic fibrosis.
CFTR-independent ATP release from epithelial cells triggered by mechanical stimuli.
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.
CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells.
CFTR-mediated Cl(-) transport in the acinar and duct cells of rabbit lacrimal gland.
CFTR-mediated inhibition of epithelial Na+ conductance in human colon is defective in cystic fibrosis.
CFTR-NHERF2-LPA? Complex in the Airway and Gut Epithelia.
CFTR-regulated chloride transport at the ocular surface in living mice measured by potential differences.
CFTR-SLC26 transporter interactions in epithelia.
CFTR: A New Horizon in the Pathomechanism and Treatment of Pancreatitis.
CFTR: cystic fibrosis and beyond.
CFTR: domains, structure, and function.
CFTR: interacting with everything?
CFTR: Ligand exchange between a permeant anion ([Au(CN)2]-) and an engineered cysteine (T338C) blocks the pore.
CFTR: mechanism of anion conduction.
CFTR: more than just a chloride channel.
CFTR: New insights into structure and function and implications for modulation by small molecules.
CFTR: the nucleotide binding folds regulate the accessibility and stability of the activated state.
CFTR: what's it like inside the pore?
cGMP inhibition of type 3 phosphodiesterase is the major mechanism by which C-type natriuretic peptide activates CFTR in the shark rectal gland.
cGMP stimulation of cystic fibrosis transmembrane conductance regulator Cl- channels co-expressed with cGMP-dependent protein kinase type II but not type Ibeta.
Challenges Facing Airway Epithelial Cell-Based Therapy for Cystic Fibrosis.
Challenges in care of adult CF patients--the specialist cystic fibrosis team.
Challenges in the use of highly effective modulator treatment for cystic fibrosis.
Changes in accessibility of cytoplasmic substances to the pore associated with activation of the cystic fibrosis transmembrane conductance regulator chloride channel.
Changes in gene expression in gills of the euryhaline killifish Fundulus heteroclitus after abrupt salinity transfer.
Changes in ion transport in inflammatory disease.
Changes in lipid raft proteome upon TNF-? stimulation of cystic fibrosis cells.
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.
Changes of chloride channels in the lacrimal glands of a rabbit model of Sjögren syndrome.
Changing incidence of cystic fibrosis in Wisconsin, USA.
Changing landscape: psychological care in the era of cystic fibrosis transmembrane conductance regulator modulators.
Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators.
Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop.
Channel-lining residues in the M3 membrane-spanning segment of the cystic fibrosis transmembrane conductance regulator.
Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia.
Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell.
Chaperoning the maturation of the cystic fibrosis transmembrane conductance regulator.
Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model.
Characteristics of Cl- uptake in rat alveolar type I cells.
Characterization and localization of the rabbit ocular calcitonin gene-related peptide (CGRP)-receptor component protein (RCP).
Characterization of 19 disease-associated missense mutations in the regulatory domain of the cystic fibrosis transmembrane conductance regulator.
Characterization of a 7,8-benzoflavone double effect on CFTR Cl(-) channel activity.
Characterization of a critical role for CFTR chloride channels in cardioprotection against ischemia/reperfusion injury.
Characterization of a disease-associated mutation affecting a putative splicing regulatory element in intron 6b of the cystic fibrosis transmembrane conductance regulator (CFTR)gene.
Characterization of a large human transgene following invasin-mediated delivery in a bacterial artificial chromosome.
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.
Characterization of a recurrent novel large duplication in the cystic fibrosis transmembrane conductance regulator gene.
Characterization of an adenovirus gene transfer vector containing an E4 deletion.
Characterization of an intron 12 splice donor mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Characterization of CFTR expression and chloride channel activity in human endothelia.
Characterization of CFTR expression in a human pulmonary mucoepidermoid carcinoma cell line, NCI-H292 cells.
Characterization of CFTR High Expresser cells in the intestine.
Characterization of clinical and genetic spectrum of Chinese patients with cystic fibrosis.
Characterization of cyclic AMP-regulated chloride conductance in the pigmented rabbit conjunctival epithelial cells.
Characterization of Defects in Ion Transport and Tissue Development in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Knockout Rats.
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.
Characterization of factors involved in modulating persistence of transgene expression from recombinant adenovirus in the mouse lung.
Characterization of ion channel and transporter mRNA expressions in isolated gill chloride and pavement cells of seawater acclimating eels.
Characterization of mitochondrial function in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function.
Characterization of more than 85% of cystic fibrosis alleles in the Greek population, including five novel mutations.
Characterization of mutations located in exon 18 of the CFTR gene.
Characterization of Nasal Potential Difference in cftr Knockout and F508del-CFTR Mice.
Characterization of oncocytes in deep esophageal glands.
Characterization of polyclonal and monoclonal antibodies to cystic fibrosis transmembrane conductance regulator.
Characterization of the adenosinetriphosphatase and transport activities of purified cystic fibrosis transmembrane conductance regulator.
Characterization of the cAMP response element of the cystic fibrosis transmembrane conductance regulator gene promoter.
Characterization of the cystic fibrosis transmembrane conductance regulator in a colonocyte cell line.
Characterization of the cystic fibrosis transmembrane conductance regulator promoter region. Chromatin context and tissue-specificity.
Characterization of the effects of Enterococcus faecium on intestinal epithelial transport properties in piglets.
Characterization of the inhibitory effect of boiled rice on intestinal chloride secretion in guinea pig crypt cells.
Characterization of the internalization pathways for the cystic fibrosis transmembrane conductance regulator.
Characterization of the ion transport responses to ADH in the MDCK-C7 cell line.
Characterization of the oligosaccharide structures associated with the cystic fibrosis transmembrane conductance regulator.
Characterization of the promoter region of the cystic fibrosis transmembrane conductance regulator gene.
Characterization of the trafficking pathway of cystic fibrosis transmembrane conductance regulator in baby hamster kidney cells.
Characterization of two distinct chloride channels in cultured dog pancreatic duct epithelial cells.
Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia.
Characterizing diverse orthologues of the cystic fibrosis transmembrane conductance regulator protein for structural studies.
Characterizing responses to CFTR-modulating drugs using rectal organoids derived from subjects with cystic fibrosis.
Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR trafficking.
Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein.
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.
Chemical rescue of deltaF508-CFTR mimics genetic repair in cystic fibrosis bronchial epithelial cells.
Chemically modified peptide scaffolds target the CFTR-associated ligand PDZ domain.
Chemotoxicity of doxorubicin and surface expression of P-glycoprotein (MDR1) is regulated by the Pseudomonas aeruginosa toxin Cif.
Chenodeoxycholic Acid Requires Activation of EGFR, EPAC and Ca2+ to Stimulate CFTR-dependent Cl- Secretion in Human Colonic T84 Cells.
Chenodeoxycholic acid stimulates Cl- secretion via cAMP signaling and increases cystic fibrosis transmembrane conductance regulator phosphorylation in T84 cells.
Children and adolescents with cystic fibrosis display moderate bone microarchitecture abnormalities: data from high-resolution peripheral quantitative computed tomography.
Chimeric constructs endow the human CFTR Cl channel with the gating behavior of murine CFTR.
Chitosan Nanocomplexes for the Delivery of ENaC Antisense Oligonucleotides to Airway Epithelial Cells.
Chloride - The Underrated Ion in Nociceptors.
Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator.
Chloride channel function is linked to epithelium-dependent airway relaxation.
Chloride channel gene expression in the rabbit cornea.
Chloride channel-targeted therapy for secretory diarrheas.
Chloride channels in apical membrane of primary cultures of rabbit distal bright convoluted tubule.
Chloride channels in stroke.
Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia.
Chloride channels in the plasma membrane of a foetal Drosophila cell line, S2.
Chloride channels in the small intestinal cell line IEC-18.
Chloride channels of intracellular membranes.
Chloride channels of intracellular organelles and their potential role in cystic fibrosis.
Chloride channels: a molecular perspective.
Chloride channels: an emerging molecular picture.
Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings.
Chloride conductance and sodium-dependent glucose transport in rat and human enterocytes.
Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.
Chloride conductance in HT29 cells: investigations with apical membrane vesicles and RT-PCR.
Chloride Conductance, Nasal Potential Difference and Cystic Fibrosis Pathophysiology.
Chloride currents activated by calcitonin and cAMP in primary cultures of rabbit distal convoluted tubule.
Chloride currents in primary cultures of rabbit proximal and distal convoluted tubules.
Chloride secretion and function of serous and mucous cells of human airway glands.
Chloride secretion by renal collecting ducts.
Chloride secretion in a morphologically differentiated human colonic cell line that expresses the epithelial Na+ channel.
Chloride secretion in the trachea of null cystic fibrosis mice: the effects of transfection with pTrial10-CFTR2.
Chloride transport in microperfused interlobular ducts isolated from guinea-pig pancreas.
Chloride transport-driven alveolar fluid secretion is a major contributor to cardiogenic lung edema.
Chloride transporting capability of Calu-3 epithelia following persistent knockdown of the cystic fibrosis transmembrane conductance regulator, CFTR.
Chloride turnover and ion-transporting activities of yolk-sac preparations (yolk balls) separated from Mozambique tilapia embryos and incubated in freshwater and seawater.
Chloride-bicarbonate exchangers in the human fetal pancreas.
Chloride-dependent secretion of alveolar wall liquid determined by optical-sectioning microscopy.
Chlorotoxin does not inhibit volume-regulated, calcium-activated and cyclic AMP-activated chloride channels.
Cholangiocyte biology and cystic fibrosis liver disease.
Cholera: something old, something new.
Cholestasis in Benign Recurrent Intrahepatic Cholestasis 2.
Cholestatic properties and hepatic transport of steroid glucuronides.
Cholesterol Interaction Directly Enhances Intrinsic Activity of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Cholesterol modulates cell signaling and protein networking by specifically interacting with PDZ domain-containing scaffold proteins.
Cholesterol modulates CFTR confinement in the plasma membrane of primary epithelial cells.
Chromanol 293B, a blocker of the slow delayed rectifier K+ current (IKs), inhibits the CFTR Cl- current.
Chromatin Dynamics in the Regulation of CFTR Expression.
Chromatin remodeling mediated by the FOXA1/A2 transcription factors activates CFTR expression in intestinal epithelial cells.
Chronic airway infection/inflammation induces a Ca2+i-dependent hyperinflammatory response in human cystic fibrosis airway epithelia.
Chronic ataluren (PTC124) treatment of nonsense mutation cystic fibrosis.
Chronic electronic cigarette exposure in mice induces features of COPD in a nicotine-dependent manner.
Chronic inflammation in the cystic fibrosis lung: alterations in inter- and intracellular signaling.
Chronic pancreatitis associated with the p.G208A variant of PRSS1 gene in a European patient.
Chronic Pancreatitis in the 21st Century - Research Challenges and Opportunities: Summary of a National Institute of Diabetes and Digestive and Kidney Diseases Workshop.
Chronic pancreatitis.
Chronic pancreatitis: challenges and advances in pathogenesis, genetics, diagnosis, and therapy.
Chronic pancreatitis: controversies in etiology, diagnosis and treatment.
Chronic pancreatitis: genetics and pathogenesis.
Chronic rhinosinusitis in patients with cystic fibrosis-Current management and new treatments.
Chronic Rhinosinusitis in Patients with Cystic Fibrosis.
Cif is negatively regulated by the TetR family repressor CifR.
Cigarette and ENDS preparations differentially regulate ion channels and mucociliary clearance in primary normal human bronchial 3D cultures.
Cigarette smoke activates CFTR through ROS-stimulated cAMP signaling in human bronchial epithelial cells.
Cigarette Smoke and CFTR: Implications in the pathogenesis of COPD.
Cigarette Smoke Exposure Induces Retrograde Trafficking of CFTR to the Endoplasmic Reticulum.
Cigarette smoke exposure reveals a novel role for the MEK/ERK1/2 MAPK pathway in regulation of CFTR.
Cigarette Smoke Induces Systemic Defects in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Function.
Cigarette Smoke-Induced Acquired Dysfunction of Cystic Fibrosis Transmembrane Conductance Regulator in the Pathogenesis of Chronic Obstructive Pulmonary Disease.
Cigarette Smoke-induced Ca2+ Release Leads to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Dysfunction.
Cigarette smoke-induced differential expression of the genes involved in exocrine function of the rat pancreas.
cis-Acting elements within CFTR 5'-flanking DNA are not sufficient to decrease gene expression in response to phorbol ester.
Cl(-) channels in apoptosis.
Cl(-) channels in smooth muscle cells.
Cl(-)-dependent HCO3- transport by cystic fibrosis transmembrane conductance regulator.
Cl- absorption across the thick ascending limb is not altered in cystic fibrosis mice. A role for a pseudo-CFTR Cl- channel.
Cl- and K+ transport in human biliary cell lines.
Cl- channel activity in Xenopus oocytes expressing the cystic fibrosis gene.
Cl- channel inhibition by glibenclamide is not specific for the CFTR-type Cl- channel.
Cl- channels regulate lipid droplet formation via Rab8a expression during adipocyte differentiation.
Cl- interference with the epithelial Na+ channel ENaC.
Cl- is required for HCO3- entry necessary for sperm capacitation in guinea pig: involvement of a Cl-/HCO3- exchanger (SLC26A3) and CFTR.
Cl- secretion by trachea of CFTR (+/-) and (-/-) fetal mouse.
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.
Cl- transport in an immortalized human epithelial cell line (NCM460) derived from the normal transverse colon.
Clara cell impact in air-side activation of CFTR in small pulmonary airways.
Clarification of laboratory and clinical variables that influence cystic fibrosis newborn screening with initial analysis of immunoreactive trypsinogen.
ClC and CFTR chloride channel gating.
CLC-0 and CFTR: chloride channels evolved from transporters.
ClC-2 contributes to native chloride secretion by a human intestinal cell line, Caco-2.
ClC-3B, a novel ClC-3 splicing variant that interacts with EBP50 and facilitates expression of CFTR-regulated ORCC.
ClC-5: ontogeny of an alternative chloride channel in respiratory epithelia.
CLCA protein and chloride transport in canine retinal pigment epithelium.
CLCA4 variants determine the manifestation of the cystic fibrosis basic defect in the intestine.
Clinical and genetic features of patients with congenital unilateral absence of the vas deferens.
Clinical and genetic risk factors for cystic fibrosis-related liver disease.
Clinical and radiological outcome of patients suffering from chronic pancreatitis associated with gene mutations.
Clinical chronic pancreatitis.
Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model.
Clinical Effectiveness of Lumacaftor/Ivacaftor in Cystic Fibrosis Patients Homozygous for F508del-CFTR.
Clinical evaluation of the infertile male with respect to genetic etiologies.
Clinical evaluation of the Nanoduct sweat test system in the diagnosis of cystic fibrosis after newborn screening.
Clinical evidence of pathogenesis in chronic pancreatitis.
Clinical Features and Endoscopic Treatment of Chinese Patients With Hereditary Pancreatitis.
Clinical genetic testing for male factor infertility: current applications and future directions.
Clinical hallmarks and genetic polymorphisms in the CFTR gene contribute to the disclosure of the A1006E mutation.
Clinical implications of cystic fibrosis transmembrane conductance regulator mutations.
Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.
Clinical outcomes in infants with cystic fibrosis transmembrane conductance regulator (CFTR) related metabolic syndrome.
Clinical phenotype and genotype of children with borderline sweat test and abnormal nasal epithelial chloride transport.
Clinical practice and genetic counseling for cystic fibrosis and CFTR-related disorders.
Clinical presentation of exclusive cystic fibrosis lung disease.
Clinically Applicable Procedure for Gene Delivery to Fetal Gut by Ultrasound-Guided Gastric Injection: Toward Prenatal Prevention of Early-Onset Intestinal Diseases.
Clinically applicable procedure for gene delivery to fetal gut by ultrasound-guided gastric injection: toward prenatal prevention of early-onset intestinal diseases.
Clinically-approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia.
Cloning and expression of a 5HT7 receptor from Xenopus laevis.
Cloning and expression of a cDNA for the human prostanoid IP receptor.
Cloning and sequence analysis of rat cystic fibrosis transmembrane conductance regulator.
Cloning the mouse homolog of the human cystic fibrosis transmembrane conductance regulator gene.
Cloning, characterization, and functional expression of a CNP receptor regulating CFTR in the shark rectal gland.
Cloning, functional expression, and characterization of the human prostaglandin E2 receptor EP2 subtype.
Clusters of Cl- channels in CFTR-expressing Sf9 cells switch spontaneously between slow and fast gating modes.
Co- and posttranslational translocation mechanisms direct cystic fibrosis transmembrane conductance regulator N terminus transmembrane assembly.
Cocoa-related flavonoids inhibit CFTR-mediated chloride transport across T84 human colon epithelia.
Coculture of Staphylococcus aureus with Pseudomonas aeruginosa Drives S. aureus towards Fermentative Metabolism and Reduced Viability in a Cystic Fibrosis Model.
Codon bias and the folding dynamics of the cystic fibrosis transmembrane conductance regulator.
Combination of Correctors Rescue ?F508-CFTR by Reducing Its Association with Hsp40 and Hsp27.
Combination of Correctors Rescues CFTR Transmembrane-Domain Mutants by Mitigating their Interactions with Proteostasis.
Combination Therapy with Cystic Fibrosis Transmembrane Conductance Regulator Modulators Augment the Airway Functional Microanatomy.
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.
Combined Computational-Experimental Analyses of CFTR Exon Strength Uncover Predictability of Exon Skipping Level.
Combined Use of CFTR Correctors in LGMD2D Myotubes Improves Sarcoglycan Complex Recovery.
Combining theoretical and experimental data to decipher CFTR 3D structures and functions.
COMMD1 modulates noxious inflammation in cystic fibrosis.
Commensal bacteria increase invasion of intestinal epithelium by Salmonella enterica serovar Typhi.
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).
Common CFTR gene variants influence body composition and survival in rural Ghana.
Common CFTR mutations are not likely to predispose to chronic bronchitis in northern Germany.
Common mutations in Cuban cystic fibrosis patients.
Compacted DNA nanoparticles administered to the nasal mucosa of cystic fibrosis subjects are safe and demonstrate partial to complete cystic fibrosis transmembrane regulator reconstitution.
Comparative analysis of common CFTR polymorphisms poly-T, TG-repeats and M470V in a healthy Chinese population.
Comparative biology of cystic fibrosis animal models.
Comparative genomic sequence analysis of the human and mouse cystic fibrosis transmembrane conductance regulator genes.
Comparative pharmacology of the activity of wild-type and G551D mutated CFTR chloride channel: effect of the benzimidazolone derivative NS004.
Comparative physical mapping of targeted regions of the rat genome.
Comparative processing and function of human and ferret cystic fibrosis transmembrane conductance regulator.
Comparison of Ambient and Atmospheric Pressure Ion Sources for Cystic Fibrosis Exhaled Breath Condensate Ion Mobility-Mass Spectrometry Metabolomics.
Comparison of Cas9 and Cas12a CRISPR editing methods to correct the W1282X-CFTR mutation.
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.
Comparison of Nasal Potential Difference and Intestinal Current Measurements as Surrogate Markers for CFTR function.
Comparison of radiographic and clinical characteristics of low-risk and high-risk cystic fibrosis genotypes.
Comparison of the bacterial HelA protein to the F508 region of the cystic fibrosis transmembrane regulator.
Comparison of the gating behaviour of human and murine cystic fibrosis transmembrane conductance regulator Cl- channels expressed in mammalian cells.
Comparison of two different protocols of neonatal screening for 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.
Comparisons of two types of teleostean pseudobranchs, silver moony (Monodactylus argenteus) and tilapia (Oreochromis mossambicus), with salinity-dependent morphology and ion transporter expression.
Compartmentalized Accumulation of cAMP near complexes of Multidrug Resistance Protein 4 (MRP4) and Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Contributes to Drug-induced Diarrhea.
Compartmentalized autocrine signaling to cystic fibrosis transmembrane conductance regulator at the apical membrane of airway epithelial cells.
Compartmentalized cyclic adenosine 3',5'-monophosphate at the plasma membrane clusters PDE3A and cystic fibrosis transmembrane conductance regulator into microdomains.
Competitive RT-PCR to quantify CFTR mRNA in human endometrium.
Complement yourself: Transcomplementation rescues partially folded mutant proteins.
Complementation of null CF mice with a human CFTR YAC transgene.
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.
Complete ascertainment of intragenic copy number mutations (CNMs) in the CFTR gene and its implications for CNM formation at other autosomal loci.
Complete cystic fibrosis transmembrane conductance regulator gene sequencing in patients with idiopathic chronic pancreatitis and controls.
Complete gene scanning by temperature gradient capillary electrophoresis using the cystic fibrosis transmembrane conductance regulator gene as a model.
Complete identification of cystic fibrosis transmembrane conductance regulator mutations in the CF population of Saguenay Lac-Saint-Jean (Quebec, Canada).
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
Complete mutational screening of the cystic fibrosis transmembrane conductance regulator gene: cystic fibrosis mutations are not involved in healthy men with reduced sperm quality.
Complex allele [-102T>A+S549R(T>G)] is associated with milder forms of cystic fibrosis than allele S549R(T>G) alone.
Complexity of phenotypes induced by p.Asn1303Lys-CFTR correlates with difficulty to rescue and activate this protein.
Composition of mineralizing incisor enamel in cystic fibrosis transmembrane conductance regulator-deficient mice.
Compound genetic abnormalities in patients with cystic fibrosis transmembrane regulator gene mutation.
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.
Comprehensive and accurate mutation scanning of the CFTR gene by two-dimensional DNA electrophoresis.
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.
Comprehensive cystic fibrosis mutation epidemiology and haplotype characterization in a southern Italian population.
Comprehensive genetic analysis of the cystic fibrosis transmembrane conductance regulator from dried blood specimens - Implications for newborn screening.
Comprehensive screening for PRSS1, SPINK1, CFTR, CTRC and CLDN2 gene mutations in Chinese paediatric patients with idiopathic chronic pancreatitis: a cohort study.
Computational Analysis of Energy Landscapes Reveals Dynamic Features That Contribute to Binding of Inhibitors to CFTR-Associated Ligand.
Computational design of a PDZ domain peptide inhibitor that rescues CFTR activity.
Computational studies reveal phosphorylation-dependent changes in the unstructured R domain of CFTR.
Computed Tomography (CT) Scanning Facilitates Early Identification of Neonatal Cystic Fibrosis Piglets.
Computed tomography correlates with improvement with ivacaftor in cystic fibrosis patients with G551D mutation.
Concentration of fractional excretion of nitric oxide (FENO): A potential airway biomarker of restored CFTR function.
Confirming the phylogeny of mammals by use of large comparative sequence data sets.
Conformation, independent of charge, in the R domain affects cystic fibrosis transmembrane conductance regulator channel openings.
Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments.
Conformational change of the extracellular parts of the CFTR protein during channel gating.
Conformational change opening the CFTR chloride channel pore coupled to ATP-dependent gating.
Conformational changes in a pore-lining helix coupled to cystic fibrosis transmembrane conductance regulator channel gating.
Conformational changes in the catalytically inactive nucleotide-binding site of CFTR.
Conformational Changes of CFTR upon Phosphorylation and ATP Binding.
Conformational Changes Relevant to Channel Activity and Folding within the first Nucleotide Binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator.
Congenital absence of the vas deferens: incomplete penetrance of cystic fibrosis gene mutations.
Congenital absence of vas deferens and cystic fibrosis.
Congenital bilateral absence of the vas deferens (CBAVD) and cystic fibrosis transmembrane regulator (CFTR): correlation between genotype and phenotype.
Congenital bilateral absence of the vas deferens (CBAVD): do genetic disorders modify assisted reproductive technologies outcomes?
Congenital bilateral absence of the vas deferens as an atypical form of cystic fibrosis: reproductive implications and genetic counseling.
Congenital bilateral absence of the vas deferens: clinical characteristics, biological parameters, cystic fibrosis transmembrane conductance regulator gene mutations, and implications for genetic counseling.
Congenital bilateral absence of vas deferens with a new missense mutation (P499A) in the CFTR gene.
Congenital Chloride-losing Diarrhea Causing Mutations in the STAS Domain Result in Misfolding and Mistrafficking of SLC26A3.
Connections Between Genetics and Clinical Data: Role of MCP-1, CFTR, and SPINK-1 in the Setting of Acute, Acute Recurrent, and Chronic Pancreatitis.
Connectivity mapping (ssCMap) to predict A20-inducing drugs and their antiinflammatory action in cystic fibrosis.
Consequences of CRISPR-Cas9-Mediated CFTR Knockout in Human Macrophages.
Conservation of CFTR codon frequency through primates suggests synonymous mutations could have a functional effect.
Conserved allosteric hot spots in the transmembrane domains of cystic fibrosis transmembrane conductance regulator (CFTR) channels and multidrug resistance protein (MRP) pumps.
Considerations for the Conduct of Clinical Trials with Antiinflammatory Agents in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report.
Constitutive activation of nuclear factor ?B contributes to cystic fibrosis transmembrane conductance regulator expression and promotes human cervical cancer progression and poor prognosis.
Constitutive internalization of cystic fibrosis transmembrane conductance regulator occurs via clathrin-dependent endocytosis and is regulated by protein phosphorylation.
Constitutive mucin secretion linked to CFTR expression.
Constrained bithiazoles: small molecule correctors of defective ?F508-CFTR protein trafficking.
Contractile Properties of Intrapulmonary Airway Smooth Muscle in Cystic Fibrosis.
Contribution of a leucine residue in the first transmembrane segment to the selectivity filter region in the CFTR chloride channel.
Contribution of CFTR to alveolar fluid clearance by lipoxin A4 via PI3K/Akt pathway in LPS-induced acute lung injury.
Contribution of concentration-sensitive sodium channels to the absorption of alveolar fluid in mice.
Contribution of M470V variant to cystic fibrosis: First study in CF and normal Tunisian population.
Contribution of proline residues in the membrane-spanning domains of cystic fibrosis transmembrane conductance regulator to chloride channel function.
Contribution of R domain phosphoserines to the function of CFTR studied in Fischer rat thyroid epithelia.
Contribution of the CFTR gene, the pancreatic secretory trypsin inhibitor gene (SPINK1) and the cationic trypsinogen gene (PRSS1) to the etiology of recurrent pancreatitis.
Contribution of the eighth transmembrane segment to the function of the CFTR chloride channel pore.
Control of cellular GADD34 levels by the 26S proteasome.
Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis.
Control of CFTR chloride conductance by ATP levels through non-hydrolytic binding.
Control of cystic fibrosis transmembrane conductance regulator expression by BAP31.
Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi.
Control of dynamic CFTR selectivity by glutamate and ATP in epithelial cells.
Control of epithelial ion transport by Cl- and PDZ proteins.
Control of epithelial Na+ conductance by the cystic fibrosis transmembrane conductance regulator.
Control of Ion Transport by Tmem16a Expressed in Murine Intestine.
Control of the CFTR channel's gates.
Control of the cystic fibrosis transmembrane conductance regulator by alphaG(i) and RGS proteins.
Controlling Allosteric Networks in Proteins.
Convective washout reduces the antidiarrheal efficacy of enterocyte surface-targeted antisecretory drugs.
Convergence of IRBIT, phosphatidylinositol (4,5) bisphosphate, and WNK/SPAK kinases in regulation of the Na+-HCO3- cotransporters family.
Converting nonhydrolyzable nucleotides to strong cystic fibrosis transmembrane conductance regulator (CFTR) agonists by gain of function (GOF) mutations.
COOH-terminal truncations promote proteasome-dependent degradation of mature cystic fibrosis transmembrane conductance regulator from post-Golgi compartments.
Cooperative assembly and misfolding of CFTR domains in vivo.
Cooperativity and flexibility of cystic fibrosis transmembrane conductance regulator transmembrane segments participate in membrane localization of a charged residue.
Coordinate upregulation of guanylin and uroguanylin expression by hypertonicity in HT29-18-N2 cells.
Coordinated release of nucleotides and mucin from human airway epithelial Calu-3 cells.
Coordination of pancreatic HCO3- secretion by protein-protein interaction between membrane transporters.
COPII machinery cooperates with ER-localized Hsp40 to sequester misfolded membrane proteins into ER-associated compartments.
COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code.
Copy number-dependent expression of a YAC-cloned human CFTR gene in a human epithelial cell line.
CORK Study in Cystic Fibrosis: Sustained Improvements in Ultra-Low-Dose Chest CT Scores After CFTR Modulation With Ivacaftor.
Coronavirus 3CLpro proteinase cleavage sites: possible relevance to SARS virus pathology.
Correcting CFTR: New Gene Editing Strategies for Rescuing CFTR Function Ex Vivo.
Correcting temperature-sensitive protein folding defects.
Correcting the F508del-CFTR variant by modulating eukaryotic translation initiation factor 3-mediated translation initiation.
Correction of aberrant splicing of the cystic fibrosis transmembrane conductance regulator (CFTR) gene by antisense oligonucleotides.
Correction of Airway Stem Cells: Genome Editing Approaches for the Treatment of Cystic Fibrosis.
Correction of cAMP-stimulated fluid secretion in cystic fibrosis airway epithelia: efficiency of adenovirus-mediated gene transfer in vitro.
Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators.
Correction of CFTR malfunction and stimulation of Ca-activated Cl channels restore HCO3- secretion in cystic fibrosis bile ductular cells.
Correction of defective protein kinesis of human P-glycoprotein mutants by substrates and modulators.
Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells.
Correction of F508del-CFTR trafficking by the sponge alkaloid latonduine is modulated by interaction with PARP.
Correction of G551D-CFTR transport defect in epithelial monolayers by genistein but not by CPX or MPB-07.
Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR.
Correction of mutations within the cystic fibrosis transmembrane conductance regulator by site-directed RNA editing.
Correction of the ?F508 Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator Gene by Zinc-Finger Nuclease Homology-Directed Repair.
Correction of the apical membrane chloride permeability defect in polarized cystic fibrosis airway epithelia following retroviral-mediated gene transfer.
Correction of the CF defect by curcumin: hypes and disappointments.
Correction of the cystic fibrosis defect by gene complementation in human intrahepatic biliary epithelial cell lines.
Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer.
Correction of the ion transport defect in cystic fibrosis transgenic mice by gene therapy.
Correction to Molecular Basis for Fe(III)-Independent Curcumin Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Activity.
Correction.
Correction: cAMP/Protein Kinase A Activates Cystic Fibrosis Transmembrane Conductance Regulator for ATP Release from Rat Skeletal Muscle during Low pH or Contractions.
Correction: Learning from each other: ABC transporter regulation by protein phosphorylation in plant and mammalian systems.
Corrector combination therapies for F508del-CFTR.
Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del).
Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis.
Correctors and Potentiators Rescue Function of the Truncated W1282X-Cystic Fibrosis Transmembrane Regulator (CFTR) Translation Product.
Correctors enhance maturation of DeltaF508 CFTR by promoting interactions between the two halves of the molecule.
Correctors promote maturation of cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by binding to the protein.
Correctors Rescue CFTR Mutations in Nucleotide-Binding Domain 1 (NBD1) by Modulating Proteostasis.
Correlates of osteopenia in patients with cystic fibrosis.
Correlating Cystic Fibrosis Transmembrane Conductance Regulator Function with Clinical Features to Inform Precision Treatment of Cystic Fibrosis.
Correlation between genotype and phenotype in patients with cystic fibrosis. The Cystic Fibrosis Genotype-Phenotype Consortium.
Correlation between nasal potential difference measurements, genotype and clinical condition in patients with cystic fibrosis.
Correlation of apical fluid-regulating channel proteins with lung function in human COPD lungs.
Correlation of sweat chloride concentration with classes of the cystic fibrosis transmembrane conductance regulator gene mutations.
Correlation of sweat chloride concentration with genotypes in cystic fibrosis patients in Saguenay Lac-Saint-Jean, Quebec, Canada.
Correlations between cystic fibrosis genotype and sinus disease severity in chronic rhinosinusitis.
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].
Corticosteroid use and increased CXCR2 levels on leukocytes are associated with lumacaftor/ivacaftor discontinuation in cystic fibrosis patients homozygous for the F508del CFTR mutation.
Corticosteroids correct aberrant CFTR localization in the duct and regenerate acinar cells in autoimmune pancreatitis.
Cortisol regulates insulin-like growth-factor binding protein (igfbp) gene expression in Atlantic salmon parr.
Cortisol regulation of ion transporter mRNA in Atlantic salmon gill and the effect of salinity on the signaling pathway.
Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro.
Could growth retardation in cystic fibrosis be partly due to deficient steroid and thyroid hormonogenesis?
Counteracting suppression of CFTR and voltage-gated K+ channels by a bacterial pathogenic factor with the natural product tannic acid.
Coupled movement of permeant and blocking ions in the CFTR chloride channel pore.
Coupled secretion of chloride and mucus in skin of Xenopus laevis: possible role for CFTR.
Coupling cystic fibrosis to endoplasmic reticulum stress: Differential role of Grp78 and ATF6.
Coupling of ATP hydrolysis with channel gating by purified, reconstituted CFTR.
Coupling of CFTR Cl- channel gating to an ATP hydrolysis cycle.
Covalent modification of the nucleotide binding domains of cystic fibrosis transmembrane conductance regulator.
Covalent modification of the regulatory domain irreversibly stimulates cystic fibrosis transmembrane conductance regulator.
COVID-19 meets Cystic Fibrosis: for better or worse?
COX2 in a euryhaline teleost, Fundulus heteroclitus: primary sequence, distribution, localization, and potential function in gills during salinity acclimation.
CpABC, a Cryptosporidium parvum ATP-binding cassette protein at the host-parasite boundary in intracellular stages.
CPX, a selective A1-adenosine-receptor antagonist, regulates intracellular pH in cystic fibrosis cells.
Critical modifier role of membrane-cystic fibrosis transmembrane conductance regulator-dependent ceramide signaling in lung injury and emphysema.
Critical role of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm.
Crofelemer for the treatment of chronic diarrhea in patients living with HIV/AIDS.
Crofelemer for the treatment of secretory diarrhea.
Crofelemer, a novel antisecretory agent approved for the treatment of HIV-associated diarrhea.
CROFELEMER, AN ANTI-SECRETORY ANTIDIARRHEAL PROANTHOCYANIDIN OLIGOMER EXTRACTED FROM CROTON LECHLERI, TARGETS TWO DISTINCT INTESTINAL CHLORIDE CHANNELS.
Crofelemer: In HIV Associated Diarrhea and Secretory Diarrhea - A Patent Perspective.
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.
Cross-species characterization of the promoter region of the cystic fibrosis transmembrane conductance regulator gene reveals multiple levels of regulation.
Crucial role for phylogenetically conserved cytoplasmic loop 3 in ABCC4 protein expression.
Cryo-EM Visualization of an Active High Open Probability CFTR Anion Channel.
Cryptorchidism-induced CFTR down-regulation results in disruption of testicular tight junctions through up-regulation of NF-?B/COX-2/PGE2.
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.
Crystal structure of the ATP-binding subunit of an ABC transporter.
Crystal structure of the cystic fibrosis transmembrane conductance regulator inhibitory factor Cif reveals novel active-site features of an epoxide hydrolase virulence factor.
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.
Crystallographic and single-particle analyses of native- and nucleotide-bound forms of the cystic fibrosis transmembrane conductance regulator (CFTR) protein.
Crystallographic characterization of the PDZ1 domain of the human Na+/H+ exchanger regulatory factor.
Cse1l is a negative regulator of CFTR-dependent fluid secretion.
CSN5 binds to misfolded CFTR and promotes its degradation.
CTCF regulates positioning of the human cystic fibrosis gene in association with a histone deacetylase.
Culture-dependent expression of Na+ conductances in airway epithelial cells.
Curcumin and genistein additively potentiate G551D-CFTR.
Curcumin cross-links cystic fibrosis transmembrane conductance regulator (CFTR) polypeptides and potentiates CFTR channel activity by distinct mechanisms.
Curcumin enhances cystic fibrosis transmembrane regulator expression by down-regulating calreticulin.
Curcumin opens cystic fibrosis transmembrane conductance regulator channels by a novel mechanism that requires neither ATP binding nor dimerization of the nucleotide-binding domains.
Curcumin stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.
Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects.
Current and emerging comorbidities in cystic fibrosis.
Current and novel therapeutic strategies for the management of cystic fibrosis.
Current concepts of immune dysregulation in cystic fibrosis.
Current concepts: host-pathogen interactions in cystic fibrosis airways disease.
Current insights into the pathogenesis of acute and chronic pancreatitis.
Current insights into the role of PKA phosphorylation in CFTR channel activity and the pharmacological rescue of cystic fibrosis disease-causing mutants.
Current status and future directions of gene and cell therapy for cystic fibrosis.
Current Status of Genetic Diagnosis Laboratories and Frequency of Genetic Variants Associated with Cystic Fibrosis through a Newborn-Screening Program in Turkey.
Current Treatment Options for Cystic Fibrosis-Related Liver Disease.
Cyanoquinolines with Independent Corrector and Potentiator Activities Restore {triangleup}F508-CFTR Chloride Channel Function in Cystic Fibrosis.
Cyclic AMP regulates bicarbonate secretion in cholangiocytes through release of ATP into bile.
Cyclic AMP-dependent protein kinase activation of cystic fibrosis transmembrane conductance regulator chloride channels in planar lipid bilayers.
Cyclic AMP-induced K+ secretion occurs independently of Cl- secretion in rat distal colon.
Cyclic AMP-mediated cyst expansion.
Cyclic Peptidyl Inhibitors against CAL/CFTR Interaction for Treatment of Cystic Fibrosis.
Cyst growth in ADPKD is prevented by pharmacological and genetic inhibition of TMEM16A in vivo.
Cysteamine re-establishes the clearance of Pseudomonas aeruginosa by macrophages bearing the cystic fibrosis-relevant F508del-CFTR mutation.
Cysteine accessibility probes timing and extent of NBD separation along the dimer interface in gating CFTR channels.
Cysteine modification reveals an allosteric inhibitory site on the CAL PDZ domain.
Cysteine residues in the nucleotide binding domains regulate the conductance state of CFTR channels.
Cysteine Scanning of CFTR's First Transmembrane Segment Reveals Its Plausible Roles in Gating and Permeation.
Cysteine string protein interacts with and modulates the maturation of the cystic fibrosis transmembrane conductance regulator.
Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis.
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.
Cysteine substitutions reveal dual functions of the amino-terminal tail in cystic fibrosis transmembrane conductance regulator channel gating.
Cysteine-independent inhibition of the CFTR chloride channel by the cysteine-reactive reagent sodium (2-sulphonatoethyl) methanethiosulphonate.
Cysteine-scanning mutagenesis provides no evidence for the extracellular accessibility of the nucleotide-binding domains of the multidrug resistance transporter P-glycoprotein.
Cystic fibrosis -- therapeutic challenge in cystic fibrosis children.
Cystic fibrosis ?f508 mutation screening in Brazilian women with altered fertility.
Cystic fibrosis affects specific cell type in sweat gland secretory coil.
Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.
Cystic fibrosis airway epithelium remodelling: involvement of inflammation.
Cystic fibrosis and bone disease: defective osteoblast maturation with the f508del mutation in cystic fibrosis transmembrane conductance regulator.
Cystic fibrosis and Chiari type I malformation: autopsy study of two infants with a rare association.
Cystic fibrosis and infertility caused by congenital bilateral absence of the vas deferens and related clinical entities.
Cystic fibrosis and innate immunity: how chloride channel mutations provoke lung disease.
Cystic fibrosis and related diseases of the pancreas.
Cystic fibrosis and renal disease.
Cystic fibrosis and renal disease: a case report.
Cystic fibrosis and survival to 40 years: a study of CFTR function.
Cystic Fibrosis and the Cells of the Airway Epithelium: What Are Ionocytes and What Do They Do?
Cystic Fibrosis and the Nervous System.
Cystic fibrosis as a cause of infertility.
Cystic fibrosis as a disease of misprocessing of the cystic fibrosis transmembrane conductance regulator glycoprotein.
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 diagnosed by state newborn screening: Or is it?
Cystic fibrosis drug ivacaftor stimulates CFTR channels at picomolar concentrations.
Cystic fibrosis enters the proteomics scene: new answers to old questions.
Cystic fibrosis epithelial cells are primed for apoptosis as a result of increased Fas (CD95).
Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells.
Cystic Fibrosis Foundation Pulmonary Guidelines. Use of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy in Patients with Cystic Fibrosis.
Cystic fibrosis from the gastroenterologist's perspective.
Cystic fibrosis gene and protein expression during fetal lung development.
Cystic fibrosis gene encodes a cAMP-dependent chloride channel in heart.
Cystic Fibrosis Gene Mutation Frequency Among a Group of Suspected Children in King Hussein Medical Center.
Cystic fibrosis gene mutation reduces epithelial cell acidification and injury in acid-perfused mouse duodenum.
Cystic fibrosis gene mutations and infertile men with primary testicular failure.
Cystic fibrosis gene mutations and polymorphisms in Saudi men with infertility.
Cystic fibrosis gene mutations deltaF508 and 394delTT in patients with chronic sinusitis in Finland.
Cystic fibrosis gene mutations detected in hereditary pancreatitis.
Cystic Fibrosis Gene Therapy in the UK and Elsewhere.
Cystic Fibrosis Gene Therapy: Looking Back, Looking Forward.
Cystic fibrosis gene.
Cystic fibrosis genotype and assessing rates of decline in pulmonary status.
Cystic fibrosis genotype and bacterial infection: a possible connection.
Cystic fibrosis hetero- and homozygosity is associated with inhibition of breast cancer growth.
Cystic Fibrosis Human Organs-on-a-Chip.
Cystic fibrosis in a boy with meconium ileus and mild clinical phenotype associated with 2183AA-G/D1152H genotype.
Cystic fibrosis in Korean children:a case report identified by a quantitative pilocarpine iontophoresis sweat test and genetic analysis.
Cystic fibrosis in South Africa: spectrum of disease and determinants of outcome.
Cystic fibrosis in Sudanese children: First report of 35 cases.
Cystic fibrosis in the era of genomic medicine.
Cystic fibrosis in the kidney: new lessons from impaired renal HCO3- excretion.
Cystic fibrosis in the mouse by targeted insertional mutagenesis.
Cystic fibrosis in Uruguay.
Cystic fibrosis infant care challenges in diagnosis and management in the era of newborn screening.
Cystic fibrosis is associated with a defect in apical receptor-mediated endocytosis in mouse and human kidney.
Cystic Fibrosis Lung Disease in the Aging Population.
Cystic Fibrosis Lung Disease Modifiers and Their Relevance in the New Era of Precision Medicine.
Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations.
Cystic fibrosis mouse model-dependent intestinal structure and gut microbiome.
Cystic fibrosis mRNA expression in rat brain: cerebral cortex and medial preoptic area.
Cystic fibrosis mutation classes in pediatric otitis media - Fickle or faulty?
Cystic fibrosis mutation detection by hybridization to light-generated DNA probe arrays.
Cystic fibrosis mutation frequencies in upstate New York.
Cystic fibrosis mutation I1234V in a Qatari lady.
Cystic fibrosis mutation screening in CBAVD patients and men with obstructive azoospermia or severe oligozoospermia.
Cystic fibrosis mutation screening in healthy men with reduced sperm quality.
Cystic fibrosis mutation testing in Italy.
Cystic fibrosis mutations and genotype-pulmonary phenotype analysis.
Cystic fibrosis mutations impair the fertilization rate of epididymal sperm from men with congenital absence of the vas deferens.
Cystic fibrosis mutations in Costa Rica.
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 mutations lead to carboxyl-terminal fragments that highlight an early biogenesis step of the cystic fibrosis transmembrane conductance regulator.
Cystic Fibrosis Mutations with Widely Variable Phenotype: The D1152H Example.
Cystic fibrosis neutrophils have normal intrinsic reactive oxygen species generation.
Cystic fibrosis newborn screening.
Cystic fibrosis newborn screening: the importance of bloodspot sample quality.
Cystic fibrosis of the pancreas: involvement of MUC6 mucin in obstruction of pancreatic ducts.
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 pathogens activate Ca2+-dependent mitogen-activated protein kinase signaling pathways in airway epithelial cells.
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 patients of minority race and ethnicity less likely eligible for CFTR modulators based on CFTR genotype.
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 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 phenotype evaluation and paternity outcome in 50 males with congenital bilateral absence of vas deferens.
Cystic fibrosis precision therapeutics: Emerging considerations.
Cystic fibrosis presentation in del. F508 and p. Tyr109Glyfs compound heterozygote CFTR state: a case report.
Cystic fibrosis presenting as acute pancreatitis and obstructive azoospermia in a young adult male with a novel mutation in the CFTR gene.
Cystic Fibrosis Presenting as Pseudo-Bartter Syndrome: An Important Diagnosis that is Missed!
Cystic fibrosis presenting as recurrent pancreatitis in a young child with a normal sweat test and pancreas divisum: a case report.
Cystic fibrosis presenting with hypokalemia and metabolic alkalosis in a previously healthy adolescent.
Cystic fibrosis presenting with persistent pneumonia --a case study of a 3-month-old adopted baby.
Cystic Fibrosis Related Diabetes - An Update.
Cystic Fibrosis Related Diabetes: a Unique Challenge in Diabetes Care.
Cystic fibrosis resources on the World Wide Web.
Cystic fibrosis respiratory tract salt concentration: An Exploratory Cohort Study.
Cystic Fibrosis Revisited - a Review Study.
Cystic fibrosis sputum: a barrier to the transport of nanospheres.
Cystic fibrosis swine fail to secrete airway surface liquid in response to inhalation of pathogens.
Cystic fibrosis transmembrane conductance regulator (ABCC7) structure.
Cystic fibrosis transmembrane conductance regulator (CFTR) activity in nasal epithelial cells from cystic fibrosis patients with severe genotypes.
Cystic fibrosis transmembrane conductance regulator (CFTR) allelic variants relate to shifts in faecal microbiota of cystic fibrosis patients.
Cystic fibrosis transmembrane conductance regulator (CFTR) and autophagy: hereditary defects in cystic fibrosis versus gluten-mediated inhibition in celiac disease.
Cystic fibrosis transmembrane conductance regulator (CFTR) and renal function.
Cystic fibrosis transmembrane conductance regulator (CFTR) anion binding as a probe of the pore.
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 transmembrane conductance regulator (CFTR) confers glibenclamide sensitivity to outwardly rectifying chloride channel (ORCC) in Hi-5 insect cells.
Cystic fibrosis transmembrane conductance regulator (CFTR) dependent cytoskeletal tension during lung organogenesis.
Cystic fibrosis transmembrane conductance regulator (CFTR) expression in human platelets: impact on mediators and mechanisms of the inflammatory response.
Cystic fibrosis transmembrane conductance regulator (CFTR) functionality is dependent on coatomer protein I (COPI).
Cystic fibrosis transmembrane conductance regulator (CFTR) gene 5T allele may protect against prostate cancer: a case-control study in Chinese Han population.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene abnormalities in Indian males with congenital bilateral absence of vas deferens & renal anomalies.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene defects in patients with primary sclerosing cholangitis.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation associated with a congenital bilateral absence of vas deferens.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations and risk for pancreatic adenocarcinoma.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in allergic bronchopulmonary aspergillosis.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Asians with chronic pulmonary disease: a pilot study.
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene mutations in North Egyptian population: implications for the genetic diagnosis in Egypt.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in pancreatitis.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene transcripts.
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 transmembrane conductance regulator (CFTR) is expressed in human bone.
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function.
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 transmembrane conductance regulator (CFTR) potentiators protect G551D but not ?F508 CFTR from thermal instability.
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) prevents apoptosis induced by hydrogen peroxide in basilar artery smooth muscle cells.
Cystic fibrosis transmembrane conductance regulator (CFTR) regulates embryonic organizer formation during zebrafish early embryogenesis.
Cystic fibrosis transmembrane conductance regulator (CFTR) regulates the production of osteoprotegerin (OPG) and prostaglandin (PG) E2 in human bone.
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Ubiquitylation as a Novel Pharmaceutical Target for 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 transmembrane conductance regulator (CFTR).
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR): CLOSED AND OPEN STATE CHANNEL MODELS.
Cystic fibrosis transmembrane conductance regulator (CFTR): Making an ion channel out of an active transporter structure.
Cystic fibrosis transmembrane conductance regulator activates water conductance in Xenopus oocytes.
Cystic fibrosis transmembrane conductance regulator activation by cAMP-independent mechanisms.
Cystic fibrosis transmembrane conductance regulator activation by roflumilast contributes to therapeutic benefit in chronic bronchitis.
Cystic fibrosis transmembrane conductance regulator activation by the solvent ethanol: implications for topical drug delivery.
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 transmembrane conductance regulator activation stimulates endosome fusion in vivo.
Cystic fibrosis transmembrane conductance regulator ameliorates lipopolysaccharide-induced acute lung injury by inhibiting autophagy through PI3K/AKT/mTOR pathway in mice.
Cystic fibrosis transmembrane conductance regulator and adenosine triphosphate.
Cystic fibrosis transmembrane conductance regulator and caveolin-1 regulate epithelial cell internalization of Pseudomonas aeruginosa.
Cystic fibrosis transmembrane conductance regulator and H+ permeability in regulation of Golgi pH.
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 transmembrane conductance regulator and obstructive azoospermia.
Cystic fibrosis transmembrane conductance regulator and pseudomonas.
Cystic fibrosis transmembrane conductance regulator and SLC26 transporters in HCO3(-) secretion by pancreatic duct cells.
Cystic fibrosis transmembrane conductance regulator and the etiology and pathogenesis of 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 transmembrane conductance regulator as a model substrate to study endoplasmic reticulum protein quality control in mammalian cells.
Cystic Fibrosis Transmembrane Conductance Regulator Attaches Tumor Suppressor PTEN to the Membrane and Promotes Anti Pseudomonas aeruginosa Immunity.
Cystic fibrosis transmembrane conductance regulator biomarkers in 'real life': can we evaluate individual efficacy of cystic fibrosis transmembrane conductance regulator therapy?
Cystic fibrosis transmembrane conductance regulator can export hyaluronan.
Cystic Fibrosis Transmembrane Conductance Regulator Channel Dysfunction in Non-cystic Fibrosis Bronchiectasis.
Cystic fibrosis transmembrane conductance regulator chloride channel blockers: Pharmacological, biophysical and physiological relevance.
Cystic fibrosis transmembrane conductance regulator Cl- channels with R domain deletions and translocations show phosphorylation-dependent and -independent activity.
Cystic fibrosis transmembrane conductance regulator contributes to reacidification of alkalinized lysosomes in RPE cells.
Cystic fibrosis transmembrane conductance regulator controls lung proteasomal degradation and nuclear factor-kappaB activity in conditions of oxidative stress.
Cystic fibrosis transmembrane conductance regulator currents in guinea pig pancreatic duct cells: inhibition by bicarbonate ions.
Cystic fibrosis transmembrane conductance regulator deficiency exacerbates islet cell dysfunction after beta-cell injury.
Cystic fibrosis transmembrane conductance regulator degradation depends on the lectins Htm1p/EDEM and the Cdc48 protein complex in yeast.
Cystic fibrosis transmembrane conductance regulator degradation: cross-talk between the ubiquitylation and SUMOylation pathways.
Cystic fibrosis transmembrane conductance regulator differentially regulates human and mouse epithelial sodium channels in Xenopus oocytes.
Cystic fibrosis transmembrane conductance regulator does not affect neutrophil migration across cystic fibrosis airway epithelial monolayers.
Cystic fibrosis transmembrane conductance regulator dysfunction and its treatment.
Cystic fibrosis transmembrane conductance regulator dysfunction in platelets drives lung hyperinflammation.
Cystic fibrosis transmembrane conductance regulator dysfunction in VIP knockout mice.
Cystic fibrosis transmembrane conductance regulator expression in human hypothalamus.
Cystic fibrosis transmembrane conductance regulator expression in human spinal and sympathetic ganglia.
Cystic fibrosis transmembrane conductance regulator facilitates ATP release by stimulating a separate ATP release channel for autocrine control of cell volume regulation.
Cystic Fibrosis Transmembrane Conductance Regulator Function in Airway Smooth Muscle. A Novel Role in Cystic Fibrosis Airway Obstruction.
Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers.
Cystic fibrosis transmembrane conductance regulator function, not TAS2R38 gene haplotypes, predict sinus surgery in children and young adults with cystic fibrosis.
Cystic fibrosis transmembrane conductance regulator functional evaluations in a G542X+/- IVS8Tn:T7/9 patient with acute recurrent pancreatitis.
Cystic fibrosis transmembrane conductance regulator gating requires cytosolic electrolytes.
Cystic fibrosis transmembrane conductance regulator gene abnormalities in patients with asthma and recurrent neutrophilic bronchitis.
Cystic fibrosis transmembrane conductance regulator gene mutation and lung cancer risk.
Cystic fibrosis transmembrane conductance regulator gene mutations and glutathione S-transferase null genotypes in cystic fibrosis patients in Brazil.
Cystic fibrosis transmembrane conductance regulator gene mutations in infertile males with congenital bilateral absence of the vas deferens.
Cystic fibrosis transmembrane conductance regulator gene mutations in severe nasal polyposis.
Cystic fibrosis transmembrane conductance regulator gene mutations: do they play a role in the aetiology of allergic bronchopulmonary aspergillosis?
Cystic fibrosis transmembrane conductance regulator gene polymorphisms in patients with primary sclerosing cholangitis.
Cystic fibrosis transmembrane conductance regulator gene screening and clinical correlation in Taiwanese males with congenital bilateral absence of the vas deferens.
Cystic fibrosis transmembrane conductance regulator gene variants are associated with autoimmune pancreatitis and slow response to steroid treatment.
Cystic Fibrosis Transmembrane Conductance Regulator Genotype, Not Circulating Catecholamines, Influences Cardiovascular Function in Patients with Cystic Fibrosis.
Cystic fibrosis transmembrane conductance regulator has an altered structure when its maturation is inhibited.
Cystic fibrosis transmembrane conductance regulator in follicular fluid and cumulus cells and its relationship with age.
Cystic fibrosis transmembrane conductance regulator in human and mouse red blood cell membranes and its interaction with ecto-apyrase.
Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise.
Cystic Fibrosis Transmembrane Conductance Regulator in Sarcoplasmic Reticulum of Airway Smooth Muscle. Implications for Airway Contractility.
Cystic fibrosis transmembrane conductance regulator in teleost fish.
Cystic fibrosis transmembrane conductance regulator in the endolymphatic sac of the rat.
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 transmembrane conductance regulator in the kidney: clues to its role?
Cystic fibrosis transmembrane conductance regulator inhibits epithelial Na+ channels carrying Liddle's syndrome mutations.
Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment.
Cystic fibrosis transmembrane conductance regulator inverts protein kinase A-mediated regulation of epithelial sodium channel single channel kinetics.
Cystic fibrosis transmembrane conductance regulator ion channel function testing in recurrent acute pancreatitis.
Cystic fibrosis transmembrane conductance regulator is an epithelial cell receptor for clearance of Pseudomonas aeruginosa from the lung.
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 Transmembrane Conductance Regulator Is Expressed in Mucin Granules from Calu-3 and Primary Human Airway Epithelial Cells.
Cystic fibrosis transmembrane conductance regulator is found within brain ventricular epithelium and choroid plexus.
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 transmembrane conductance regulator is involved in airway epithelial wound repair.
Cystic fibrosis transmembrane conductance regulator is involved in polyphenol-induced swelling of the endothelial glycocalyx.
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 transmembrane conductance regulator is vital to sperm fertilizing capacity and male fertility.
Cystic fibrosis transmembrane conductance regulator knockout mice exhibit aberrant gastrointestinal microbiota.
Cystic fibrosis transmembrane conductance regulator mediates sulphonylurea block of the inwardly rectifying K+ channel Kir6.1.
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 transmembrane conductance regulator mediates the cyclic adenosine monophosphate-induced fluid secretion but not the inhibition of resorption in mouse gallbladder epithelium.
Cystic fibrosis transmembrane conductance regulator modulates acute lung injury: evidence from a genetic association study*.
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 transmembrane conductance regulator modulates neurosecretory function in pulmonary neuroendocrine cell-related tumor cell line models.
Cystic fibrosis transmembrane conductance regulator modulates synaptic chloride homeostasis in motoneurons of the rat spinal cord during neonatal development.
Cystic fibrosis transmembrane conductance regulator modulation by the tobacco smoke toxin acrolein.
Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy: A Review for the Otolaryngologist.
Cystic fibrosis transmembrane conductance regulator modulators for cystic fibrosis: a new dawn?
Cystic fibrosis transmembrane conductance regulator modulators for personalized drug treatment of cystic fibrosis: progress to date.
Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives.
Cystic fibrosis transmembrane conductance regulator modulators reduce the risk of recurrent acute pancreatitis among adult patients with pancreas sufficient cystic fibrosis.
Cystic fibrosis transmembrane conductance regulator modulators: precision medicine in cystic fibrosis.
Cystic fibrosis transmembrane conductance regulator modulators: Present and future in cystic fibrosis treatment. A review.
Cystic fibrosis transmembrane conductance regulator modulators: the end of the beginning.
Cystic fibrosis transmembrane conductance regulator mutant F508del membrane expression rescues epithelial HCO3- secretion in murine intestine.
Cystic fibrosis transmembrane conductance regulator mutation spectrum in patients with cystic fibrosis in Tunisia.
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 transmembrane conductance regulator mutations at a referral center for cystic fibrosis.
Cystic fibrosis transmembrane conductance regulator mutations in azoospermic and oligospermic men and their partners.
Cystic fibrosis transmembrane conductance regulator mutations that disrupt nucleotide binding.
Cystic Fibrosis Transmembrane Conductance Regulator Potentiation as a Therapeutic Strategy for Pulmonary Edema: A Proof-of-Concept Study in Pigs.
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 transmembrane conductance regulator protein expression in brain.
Cystic fibrosis transmembrane conductance regulator protein expression in the male excretory duct system during development.
Cystic fibrosis transmembrane conductance regulator protein expression rate in healthy spermatozoa is not correlated with ovum fertilisation rate.
Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis.
Cystic fibrosis transmembrane conductance regulator protein: what is its role in cystic fibrosis?
Cystic Fibrosis Transmembrane Conductance Regulator Recruitment to Phagosomes in Neutrophils.
Cystic Fibrosis Transmembrane Conductance Regulator Reduces Microtubule-Dependent Campylobacter jejuni Invasion.
Cystic fibrosis transmembrane conductance regulator regulates epithelial cell response to Aspergillus and resultant pulmonary inflammation.
Cystic fibrosis transmembrane conductance regulator regulates luminal Cl-/HCO3- exchange in mouse submandibular and pancreatic ducts.
Cystic fibrosis transmembrane conductance regulator splice variants are not conserved and fail to produce chloride channels.
Cystic fibrosis transmembrane conductance regulator trafficking is mediated by the COPI coat in epithelial cells.
Cystic fibrosis transmembrane conductance regulator trafficking modulates the barrier function of airway epithelial cell monolayers.
Cystic fibrosis transmembrane conductance regulator with a shortened R domain rescues the intestinal phenotype of CFTR-/- mice.
Cystic fibrosis transmembrane conductance regulator-associated ATP release is controlled by a chloride sensor.
Cystic fibrosis transmembrane conductance regulator-dependent bicarbonate entry controls rat cardiomyocyte ATP release via pannexin1 through mitochondrial signalling and caspase activation.
Cystic fibrosis transmembrane conductance regulator-dependent up-regulation of Kir1.1 (ROMK) renal K+ channels by the epithelial sodium channel.
Cystic fibrosis transmembrane conductance regulator-emerging regulator of cancer.
Cystic Fibrosis Transmembrane Conductance Regulator-independent Phagosomal Acidification in Macrophages.
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 transmembrane conductance regulator-modifying medications: the future of cystic fibrosis treatment.
Cystic fibrosis transmembrane conductance regulator-mRNA delivery: a novel alternative for cystic fibrosis gene therapy.
Cystic Fibrosis Transmembrane Conductance Regulator. Implications in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Cystic fibrosis transmembrane conductance regulator. Permeant ions find the pore.
Cystic fibrosis transmembrane conductance regulator. Physical basis for lyotropic anion selectivity patterns.
Cystic fibrosis transmembrane conductance regulator. Structure and function of an epithelial chloride channel.
Cystic fibrosis transmembrane conductance regulator: a chloride channel gated by ATP binding and hydrolysis.
Cystic fibrosis transmembrane conductance regulator: a chloride channel with novel regulation.
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 Transmembrane Conductance Regulator: A Possible New Target for Photodynamic Therapy Enhances Wound Healing.
Cystic fibrosis transmembrane conductance regulator: Actin(g) as a master regulator of cholangiocyte function.
Cystic fibrosis transmembrane conductance regulator: expression and helicity of a double membrane-spanning segment.
Cystic fibrosis transmembrane conductance regulator: nucleotide binding to a synthetic peptide.
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 transmembrane conductance regulator: temperature-dependent cysteine reactivity suggests different stable conformers of the conduction pathway.
Cystic fibrosis transmembrane conductance regulator: the first nucleotide binding fold targets the membrane with retention of its ATP binding function.
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 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 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 transmembrane conductance regulators (CFTR) in biliary epithelium of patients with hepatolithiasis.
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 transmembrane regulator (CFTR) in human trophoblast BeWo cells and its relation to cell migration.
Cystic fibrosis transmembrane regulator correctors and potentiators.
Cystic fibrosis transmembrane regulator fragments with the Phe508 deletion exert a dual allosteric control over the master kinase CK2.
Cystic fibrosis transmembrane regulator gene (CFTR) is associated with abnormal enamel formation.
Cystic fibrosis transmembrane regulator gene carrier status is a risk factor for young onset pancreatic adenocarcinoma.
Cystic fibrosis transmembrane regulator gene mutations in Bahrain.
Cystic fibrosis transmembrane regulator haplotypes in households of patients with cystic fibrosis.
Cystic fibrosis transmembrane regulator inhibitors CFTR(inh)-172 and GlyH-101 target mitochondrial functions, independently of chloride channel inhibition.
Cystic fibrosis transmembrane regulator missing the first four transmembrane segments increases wild type and DeltaF508 processing.
Cystic Fibrosis Transmembrane Regulator Modulators: Implications for the Management of Depression and Anxiety in Cystic Fibrosis.
Cystic fibrosis transmembrane regulator mRNA expression relative to ion-nutrient transport in spontaneously differentiating human intestinal CaCo-2 epithelial cells.
Cystic fibrosis transmembrane regulator mutations and pancreatic disease: closing the gap between genotype and phenotype.
Cystic fibrosis transmembrane regulator potentiators as promising cystic fibrosis therapies.
Cystic fibrosis transmembrane regulator protein mutations: 'class' opportunity for novel drug innovation.
Cystic fibrosis transmembrane regulator regulates uptake of sphingoid base phosphates and lysophosphatidic acid: modulation of cellular activity of sphingosine 1-phosphate.
Cystic fibrosis with non-G551D gating mutations in Italy: Epidemiology and clinical characteristics.
Cystic fibrosis with three mutations in the cystic fibrosis transmembrane conductance regulator gene.
Cystic fibrosis year in review 2018, part 1.
Cystic fibrosis year in review 2018, part 2.
Cystic fibrosis year in review 2019: Section 1 CFTR modulators.
Cystic fibrosis year in review 2019: Section 3 multisystem-based care and research.
Cystic Fibrosis, CFTR, and Colorectal Cancer.
Cystic Fibrosis, Cystic Fibrosis Transmembrane Conductance Regulator and Drugs: Insights from Cellular Trafficking.
Cystic fibrosis, the CFTR, and rectifying Cl- channels.
Cystic fibrosis--a strategy for the future.
Cystic fibrosis--what are the prospects for a cure?
Cystic Fibrosis-Associated Liver Disease in Lung Transplant Recipients.
Cystic fibrosis-associated mutations at arginine 347 alter the pore architecture of CFTR. Evidence for disruption of a salt bridge.
Cystic fibrosis-related diabetes is caused by islet loss and inflammation.
Cystic Fibrosis-Related Diabetes.
Cystic Fibrosis-Related Diabetes: Pathophysiology and Therapeutic Challenges.
Cystic Fibrosis-related Liver Disease: The Next Challenge.
Cystic fibrosis-related oxidative stress and intestinal lipid disorders.
Cystic Fibrosis-Related Pancreatic Cysts Decrease in Size and Number Upon Treatment With Cystic Fibrosis Transmembrane Conductance Regulator Modulators.
Cystic fibrosis-style changes in the early phase of pancreatitis.
Cystic fibrosis-type mutational analysis in the ATP-binding cassette transporter signature of human P-glycoprotein MDR1.
Cystic fibrosis.
Cystic Fibrosis.
Cystic fibrosis. 4. Abnormalities of airway epithelial function and the implications of the discovery of the cystic fibrosis gene.
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: a disease of altered protein folding.
Cystic fibrosis: A disorder with defective autophagy.
Cystic fibrosis: a model system for precision medicine.
Cystic fibrosis: a mucosal immunodeficiency syndrome.
Cystic fibrosis: a new target for 4-Imidazo[2,1-b]thiazole-1,4-dihydropyridines.
Cystic Fibrosis: A Novel Pharmacologic Approach to Cystic Fibrosis Transmembrane Regulator Modulation Therapy.
Cystic Fibrosis: A Simple and Customized Strategy for Genetic Screening Able to Detect Over 90% of Identified Mutated Alleles in Brazilian Newborns.
Cystic fibrosis: An inherited disease affecting mucin-producing organs.
Cystic fibrosis: an overview.
Cystic fibrosis: basic chemical and cellular mechanisms.
Cystic fibrosis: basic science.
Cystic fibrosis: can epithelial function be restored?
Cystic fibrosis: CFTR correctors to the rescue.
Cystic Fibrosis: Emergence of Highly Effective Targeted Therapeutics and Potential Clinical Implications.
Cystic fibrosis: Evidence for gut inflammation.
Cystic fibrosis: exploiting its genetic basis in the hunt for new therapies.
Cystic fibrosis: genotypic and phenotypic variations.
Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis.
Cystic fibrosis: need for mass deployable screening methods.
Cystic fibrosis: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases.
Cystic Fibrosis: Overview of the Current Development Trends and Innovative Therapeutic Strategies.
Cystic fibrosis: pathogenesis and future treatment strategies.
Cystic Fibrosis: Pathophysiology of Lung Disease.
Cystic fibrosis: Physiopathology and the latest pharmacological treatments.
Cystic fibrosis: recent structural insights.
Cystic fibrosis: review of the decade.
Cystic fibrosis: S158N (605G --> A) is a rare genetic variant found in coupling with deltaF508.
Cystic fibrosis: the impact of analytical technology for genotype-phenotype studies.
Cystic Fibrosis: Translating Molecular Mechanisms into Effective Therapies.
Cystic-fibrosis-like disease unrelated to the cystic fibrosis transmembrane conductance regulator.
Cytochrome P450 3A4 induction: lumacaftor versus ivacaftor potentially resulting in significantly reduced plasma concentration of ivacaftor.
Cytogenetic analysis of azoospermic patients: karyotype comparison of peripheral blood lymphocytes and testicular tissue.
Cytokine dysregulation in activated cystic fibrosis (CF) peripheral lymphocytes.
Cytokine pattern in cystic fibrosis patients during antibiotic therapy and gene therapy using adenoviral vector.
Cytokine secretion by cystic fibrosis airway epithelial cells.
Cytokine-Regulation of Na(+)-K(+)-Cl(-) Cotransporter 1 and Cystic Fibrosis Transmembrane Conductance Regulator-Potential Role in Pulmonary Inflammation and Edema Formation.
Cytokine-stimulated nitric oxide production inhibits adenylyl cyclase and cAMP-dependent secretion in cholangiocytes.
Cytological characterization of apatitic calcium phosphate structures in bronchial epithelial tissue cultured from a child with cystic fibrosis (deltaF508).
Cytoplasmic loop three of cystic fibrosis transmembrane conductance regulator contributes to regulation of chloride channel activity.
Cytoplasmic pathway followed by chloride ions to enter the CFTR channel pore.
Cytoprotective antioxidant function of tyrosine and tryptophan residues in transmembrane proteins.
Cytoskeleton and CFTR.
Cytoskeleton regulators CAPZA2 and INF2 associate with CFTR to control its plasma membrane levels under EPAC1 activation.
Cytosolic pH regulates GCl through control of phosphorylation states of CFTR.
Cytosolic potassium controls CFTR deactivation in human sweat duct.
Dab2 is a key regulator of endocytosis and post-endocytic trafficking of the cystic fibrosis transmembrane conductance regulator.
Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor.
Dataset of differential lipid raft and global proteomes of SILAC-labeled cystic fibrosis cells upon TNF -? stimulation.
DCPIB is a novel selective blocker of I(Cl,swell) and prevents swelling-induced shortening of guinea-pig atrial action potential duration.
Deactivation of CFTR-Cl conductance by endogenous phosphatases in the native sweat duct.
Decoding f508del misfolding in cystic fibrosis.
Decreased expression of cystic fibrosis transmembrane conductance regulator impairs sperm quality in aged men.
Decreased expression of peroxisome proliferator activated receptor gamma in cftr-/- mice.
Decreased expression of the CFTR protein in remodeled human nasal epithelium from non-cystic fibrosis patients.
Decreased expression of the cystic fibrosis transmembrane conductance regulator protein in remodeled airway epithelium from lung transplanted patients.
Decreased Peroxisome Proliferator Activated Receptor alpha Is Associated with Bile Duct Injury in Cystic Fibrosis Transmembrane Conductance Regulator-/- Mice.
Decreased soluble adenylyl cyclase activity in cystic fibrosis is related to defective apical bicarbonate exchange and affects ciliary beat frequency regulation.
Deep interactome profiling of membrane proteins by co-interacting protein identification technology.
Defective CFTR apical endocytosis and enterocyte brush border in myosin VI-deficient mice.
Defective CFTR Expression and Function Are Detectable in Blood Monocytes: Development of a New Blood Test for Cystic Fibrosis.
Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition.
Defective CFTR leads to aberrant ?-catenin activation and kidney fibrosis.
Defective CFTR promotes intestinal proliferation via inhibition of the hedgehog pathway during cystic fibrosis.
Defective CFTR-dependent CREB activation results in impaired spermatogenesis and azoospermia.
Defective cholinergic Cl(-) secretion and detection of K(+) secretion in rectal biopsies from cystic fibrosis patients.
Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis.
Defective exocytosis and processing of insulin in a cystic fibrosis mouse model.
Defective fluid transport by cystic fibrosis airway epithelia.
Defective formation of PKA/CnA-dependent annexin 2-S100A10/CFTR complex in DeltaF508 cystic fibrosis cells.
Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein.
Defective Innate Immunity and Hyper-Inflammation in Newborn CFTR-Knockout Ferret Lungs.
DEFECTIVE ORGANELLAR ACIDIFICATION AS A CAUSE OF CYSTIC FIBROSIS LUNG DISEASE - RE-EXAMINATION OF A RECURRING HYPOTHESIS.
Defective regulation of outwardly rectifying Cl- channels by protein kinase A corrected by insertion of CFTR.
Defective regulatory volume decrease in human cystic fibrosis tracheal cells because of altered regulation of intermediate conductance Ca2+-dependent potassium channels.
Defective trafficking and function of KATP channels caused by a sulfonylurea receptor 1 mutation associated with persistent hyperinsulinemic hypoglycemia of infancy.
Defects in Gallbladder Emptying and Bile Acid Homeostasis in Mice with Cystic Fibrosis Transmembrane Conductance Regulator Deficiencies.
Defects in processing and trafficking of cystic fibrosis transmembrane conductance regulator.
Defects in processing and trafficking of the cystic fibrosis transmembrane conductance regulator.
Defining a mutational panel and predicting the prevalence of cystic fibrosis in oman.
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene.
Defining the role of CFTR channel blocker and ClC-2 activator in DNBS induced gastrointestinal inflammation.
Definition of a "functional R domain" of the cystic fibrosis transmembrane conductance regulator.
Deformation-induced ATP release from red blood cells requires CFTR activity.
Degradation of CFTR by the ubiquitin-proteasome pathway.
Dehydrated patient without clinically evident cause: A case report.
Dehydrocostuslactone, a sesquiterpene lactone activates wild-type and ?F508 mutant CFTR chloride channel.
Delayed association of the NADPH oxidase complex with macrophage vacuoles containing the opportunistic pathogen Burkholderia cenocepacia.
Delayed neutrophil apoptosis enhances NET formation in cystic fibrosis.
Deleterious impact of Pseudomonas aeruginosa on cystic fibrosis transmembrane conductance regulator function and rescue in airway epithelial cells.
Deletion and insertion mutations in short tandem repeats in the coding regions of human genes.
Deletion of cftr Leads to an Excessive Neutrophilic Response and Defective Tissue Repair in a Zebrafish Model of Sterile Inflammation.
Deletion of exons 16-17b of CFTR is frequently identified in Korean patients with 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.
Deletion of phenylalanine 508 causes attenuated phosphorylation-dependent activation of CFTR chloride channels.
Deletion of Phenylalanine 508 in the First Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator Increases Conformational Exchange and Inhibits Dimerization.
Deletion of the ubiquitin ligase Nedd4L in lung epithelia causes cystic fibrosis-like disease.
Delivery of mRNA Therapeutics for the Treatment of Hepatic Diseases.
Delivery of purified, functional CFTR to epithelial cells in vitro using influenza hemagglutinin.
Delta F508 CFTR localizes in the endoplasmic reticulum-Golgi intermediate compartment in cystic fibrosis cells.
Delta F508 CFTR pool in the endoplasmic reticulum is increased by calnexin overexpression.
Delta F508 mutation of cystic fibrosis gene is not found in chronic bronchitis with severe obstruction in Japan.
DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers.
DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis.
DeltaF508 mutation results in impaired gastric acid secretion.
Demographics of the UK cystic fibrosis population: implications for neonatal screening.
Demonstration of phosphoryl-group transfer indicates that the ABC transporter CFTR exhibits adenylate kinase activity.
Demonstration that CFTR is a chloride channel by alteration of its anion selectivity.
Denaturing high performance liquid chromatography (DHPLC) used in the detection of germline and somatic mutations.
Denopamine stimulates alveolar fluid clearance via cystic fibrosis transmembrane conductance regulator in rat lungs.
Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function.
Dependence of cAMP meditated increases in Cl(-) and HCO(3)(-) permeability on CFTR in bovine corneal endothelial cells.
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.
Deregulated expression and function of CFTR and Cl- secretion after activation of the Ras and Src/PyMT pathways in Caco-2 cells.
Derivation of adult canine intestinal organoids for translational research in gastroenterology.
Derivation of lung epithelium from human cord blood-derived mesenchymal stem cells.
Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutants.
Description of a symptomless cystic fibrosis L346P/M348K compound heterozygous Cypriot individual.
Design and characterization of a membrane protein unfolding platform in lipid bilayers.
Designing Clinical Trials for Anti-Inflammatory Therapies in Cystic Fibrosis.
Destabilization of the transmembrane domain induces misfolding in a phenotypic mutant of cystic fibrosis transmembrane conductance regulator.
Detailed analysis of structures and formulations of cationic lipids for efficient gene transfer to the lung.
DETANO and nitrated lipids increase chloride secretion across lung airway cells.
Detecting Common CFTR Mutations by Reverse Dot Blot Hybridization Method in Cystic Fibrosis First Report from Northern Iran.
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.
Detection of a de novo R1066H mutation in an Italian patient affected by cystic fibrosis.
Detection of adenylate cyclase-coupled receptors in Xenopus oocytes by coexpression with cystic fibrosis transmembrane conductance regulator.
Detection of an Apparent Homozygous 3120G>A Cystic Fibrosis Mutation on a Routine Carrier Screen.
Detection of CFTR gene mutations in patients suffering from chronic bronchitis.
Detection of CFTR mutations using ARMS and low-density microarrays.
Detection of CFTR mutations using temporal temperature gradient gel electrophoresis.
Detection of CFTR protein in human leukocytes by flow cytometry.
Detection of cystic fibrosis mutations by peptide mass signature genotyping.
Detection of Cystic Fibrosis Serological Biomarkers Using a T7 Phage Display Library.
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.
Detection of cystic fibrosis transmembrane conductance regulator ?F508 gene mutation using a paper-based nucleic acid hybridization assay and a smartphone camera.
Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials.
Detection of delta F508 mutation of the cystic fibrosis gene by matrix-assisted laser desorption/ionization mass spectrometry.
Detection of F508del mutation in cystic fibrosis transmembrane conductance regulator gene mutation among Malays.
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).
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.
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.
Detection of modifier loci influencing the lung phenotype of cystic fibrosis knockout mice.
Detection of more than 50 different CFTR mutations in a large group of German cystic fibrosis patients.
Detection of novel CFTR mutations in Taiwanese cystic fibrosis patients.
Detection of point mutations by capillary electrophoresis in liquid polymers in temporal thermal gradients.
Detection of the deltaF508 (F508del) mutation of the cystic fibrosis gene by surface plasmon resonance and biosensor technology.
Detection of the DeltaF508 mutation in the CFTR gene by means of time-resolved fluorescence methods.
Detergent binding explains anomalous SDS-PAGE migration of membrane proteins.
Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods.
Determination of the functional unit of the cystic fibrosis transmembrane conductance regulator chloride channel. One polypeptide forms one pore.
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.
Determining the pathogenicity of CFTR missense variants: Multiple comparisons of in silico predictors and variant annotation databases.
Developing novel antisecretory drugs to treat infectious diarrhea.
Development and analysis of recombinant adenoviruses for gene therapy of cystic fibrosis.
Development and characterization of secretin-stimulated secretion of cultured rat cholangiocytes.
Development and characterization of synthetic antibodies binding to the cystic fibrosis conductance regulator.
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.
Development and functional characterization of extrahepatic cholangiocyte lines from normal rats.
Development and regulation of chloride homeostasis in the central nervous system.
Development and validation of a screening test for 12 common mutations of the cystic fibrosis CFTR gene.
Development and Validation of HTS Flux Assay for Endogenously Expressed Chloride Channels in a CHO-K1 Cell Line.
DEVELOPMENT OF A POLARIZED PANCREATIC DUCTULAR CELL EPITHELIA FOR PHYSIOLOGICAL STUDIES.
Development of allele-specific multiplex PCR to determine the length of poly-T in intron 8 of CFTR.
Development of an epithelium-specific expression cassette with human DNA regulatory elements for transgene expression in lung airways.
Development of Automated Patch Clamp Technique to Investigate CFTR Chloride Channel Function.
Development of CFTR Structure.
Development of elexacaftor - tezacaftor - ivacaftor: Highly effective CFTR modulation for the majority of people with Cystic Fibrosis.
Development of intestinal ion-transporting mechanisms during smoltification and seawater acclimation in Atlantic salmon Salmo salar.
Development of PEGylated PLGA nanoparticle for controlled and sustained drug delivery in cystic fibrosis.
Development of structural marker peptides for cystic fibrosis transmembrane conductance regulator in cell plasma membrane by reversed-footprinting mass spectrometry.
Development of substituted Benzo[c]quinolizinium compounds as novel activators of the cystic fibrosis chloride channel.
Development, multiplexing, and application of ARMS tests for common mutations in the CFTR gene.
Developmental differences of cystic fibrosis transmembrane conductance regulator functional expression in isolated rat fetal distal airway epithelial cells.
Developmental expression of a mucinlike glycoprotein (MUCLIN) in pancreas and small intestine of CF mice.
Developmental expression patterns of CFTR in ferret tracheal surface airway and submucosal gland epithelia.
Developmental paradigm for early features of cystic fibrosis.
Developmental regulation of CFTR expression during human nephrogenesis.
Diabetic cornea wounds produce significantly weaker electric signals that may contribute to impaired healing.
Diagnosis and Management of Chronic Pancreatitis: A Review.
Diagnosis of cystic fibrosis in adulthood and eligibility for novel CFTR modulator therapy.
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.
Diagnosis, follow-up and treatment of cystic fibrosis-related liver disease.
Diagnostic contribution of molecular analysis of the cystic fibrosis transmembrane conductance regulator gene in patients suspected of having mild or atypical cystic fibrosis.
Diagnostic Testing in Cystic Fibrosis.
Diagnostic tools and CFTR functional assays in cystic fibrosis: utility and availability in Switzerland.
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.
Diarrhea induction by rotavirus NSP4 in the homologous mouse model system.
Dibasic protein kinase A sites regulate bursting rate and nucleotide sensitivity of the cystic fibrosis transmembrane conductance regulator chloride channel.
Diet-Induced Dysbiosis and Genetic Background Synergize With Cystic Fibrosis Transmembrane Conductance Regulator Deficiency to Promote Cholangiopathy in Mice.
Dietary genistein stimulates anion secretion across female murine intestine.
Diethylstilbestrol, a Novel ANO1 Inhibitor, Exerts an Anticancer Effect on Non-Small Cell Lung Cancer via Inhibition of ANO1.
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.
Differences between cystic fibrosis transmembrane conductance regulator and HisP in the interaction with the adenine ring of ATP.
Differences in gene mutations between Chinese and Caucasian cystic fibrosis patients.
Differences in the protein-kinase-A-dependent regulation of CFTR Cl- channels and Na+-K+ pumps in guinea-pig ventricular myocytes.
Different activation mechanisms of cystic fibrosis transmembrane conductance regulator expressed in Xenopus laevis oocytes.
Different CFTR mutational spectrum in alcoholic and idiopathic chronic pancreatitis?
Different cystic fibrosis transmembrane conductance regulator mutations in chinese men with congenital bilateral absence of vas deferens and other acquired obstructive azoospermia.
Different functional roles for K+ channel subtypes in regulating small intestinal glucose and ion transport.
Different immunohistochemical localization for TMEM16A and CFTR in acinar and ductal cells of rat major salivary glands and exocrine pancreas.
Different p97/VCP complexes function in retrotranslocation step of mammalian Er-associated degradation (ERAD).
Different SUMO paralogues determine the fate of wild-type and mutant CFTRs: biogenesis versus degradation.
Differential acidic pH sensitivity of delta F508 CFTR Cl- channel activity in lipid bilayers.
Differential activation of the HCO(3) conductance through the cystic fibrosis transmembrane conductance regulator anion channel by genistein and forskolin in murine duodenum.
Differential cellular expression of cystic fibrosis transmembrane regulator in human reproductive tissues. Clues for the infertility in patients with cystic fibrosis.
Differential contribution of cis-regulatory elements to higher order chromatin structure and expression of the CFTR locus.
Differential coupling of G protein alpha subunits to seven-helix receptors expressed in Xenopus oocytes.
Differential effects of aldosterone and vasopressin on chloride fluxes in transimmortalized mouse cortical collecting duct cells.
Differential effects of Matrigel and its components on functional activity of CFTR and ENaC in mouse endometrial epithelial cells.
Differential effects of polycationic protein on Cl- secretory and Na+ absorptive airways.
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.
Differential expression and localization of CFTR and ENaC in mouse endometrium during pre-implantation.
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.
Differential expression of intestinal ion transporters and water channel aquaporins in young piglets challenged with enterotoxigenic
Differential function of the two nucleotide binding domains on cystic fibrosis transmembrane conductance regulator.
Differential interactions of nucleotides at the two nucleotide binding domains of the cystic fibrosis transmembrane conductance regulator.
Differential localization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis airway epithelium.
Differential regulation of CFTRDeltaF508 degradation by ubiquitin ligases gp78 and Hrd1.
Differential regulation of Cl- transport proteins by PKC in Calu-3 cells.
Differential regulation of cystic fibrosis transmembrane conductance regulator and Na+,K+ -ATPase in gills of striped bass, Morone saxatilis: effect of salinity and hormones.
Differential regulation of cystic fibrosis transmembrane conductance regulator by interferon gamma in mast cells and epithelial cells.
Differential regulation of inflammation by inflammatory mediators in cystic fibrosis lung epithelial cells.
Differential regulation of single CFTR channels by PP2C, PP2A, and other phosphatases.
Differential regulations between adenosine triphosphate (ATP)- and uridine triphosphate-induced Cl(-) secretion in bovine tracheal epithelium. Direct stimulation of P1-like receptor by ATP.
Differential sensitivity of plant and yeast MRP (ABCC)-mediated organic anion transport processes towards sulfonylureas.
Differential sensitivity of the cystic fibrosis (CF)-associated mutants G551D and G1349D to potentiators of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel.
Differential stimulation of cytosolic phospholipase A2 by bradykinin in human cystic fibrosis cell lines.
Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR.
Differentiation of adult rat bone marrow stem cells into epithelial progenitor cells in culture.
Differentiation of human bronchial epithelial cells: role of hydrocortisone in development of ion transport pathways involved in mucociliary clearance.
Differentiation of immortalized epithelial cells derived from cystic fibrosis airway submucosal glands.
Diffusional mobility of the cystic fibrosis transmembrane conductance regulator mutant, delta F508-CFTR, in the endoplasmic reticulum measured by photobleaching of GFP-CFTR chimeras.
Dimeric cystic fibrosis transmembrane conductance regulator exists in the plasma membrane.
Diminished self-chaperoning activity of the DeltaF508 mutant of CFTR results in protein misfolding.
Dinucleotide (CA/GT) repeat polymorphism in intron 17B of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Direct action of genistein on CFTR.
Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-diallyl-8-cyclohexylxanthine (DAX).
Direct and indirect effects of mutations at the outer mouth of the cystic fibrosis transmembrane conductance regulator chloride channel pore.
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.
Direct block of the cystic fibrosis transmembrane conductance regulator Cl(-) channel by butyrate and phenylbutyrate.
Direct block of the cystic fibrosis transmembrane conductance regulator Cl(-) channel by niflumic acid.
Direct comparison of the functional roles played by different transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Direct effects of 9-anthracene compounds on cystic fibrosis transmembrane conductance regulator gating.
Direct interaction of a CFTR potentiator and a CFTR corrector with phospholipid bilayers.
Direct interaction with filamins modulates the stability and plasma membrane expression of CFTR.
Direct Measurement of Trafficking of the Cystic Fibrosis Transmembrane Conductance Regulator to the Cell Surface and Binding to a Chemical Chaperone.
Direct sensing of intracellular pH by the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel.
Direct visualization of cystic fibrosis transmembrane regulator mutations in the clinical laboratory setting.
Directed differentiation of cholangiocytes from human pluripotent stem cells.
Discovery and development of antisecretory drugs for treating diarrheal diseases.
Discovery of 1,2,3,4-tetrahydroisoquinoline-3-carboxylic acid diamides that increase CFTR mediated chloride transport.
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.
Discovery of ABBV/GLPG-3221, a Potent Corrector of CFTR for the Treatment of Cystic Fibrosis.
Discovery of alpha-aminoazaheterocycle-methylglyoxal adducts as a new class of high-affinity inhibitors of cystic fibrosis transmembrane conductance regulator chloride channels.
Discovery of Clinically Approved Agents That Promote Suppression of CFTR Nonsense Mutations.
Discovery of GLPG2451, a Novel Once Daily Potentiator for the Treatment of Cystic Fibrosis.
Discovery of glycine hydrazide pore-occluding CFTR inhibitors: mechanism, structure-activity analysis, and in vivo efficacy.
Discovery of Icenticaftor (QBW251), a Cystic Fibrosis Transmembrane Conductance Regulator Potentiator with Clinical Efficacy in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Discovery of Multitarget Agents Active as Broad-Spectrum Antivirals and Correctors of Cystic Fibrosis Transmembrane Conductance Regulator for Associated Pulmonary Diseases.
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.
Discovery of novel VX-809 hybrid derivatives as F508del-CFTR correctors by molecular modeling, chemical synthesis and biological assays.
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.
Discrimination between cystic fibrosis and CFTR-corrected epithelial cells by a membrane potential-sensitive probe.
Disease causing mutations in the cystic fibrosis transmembrane conductance regulator determine the functional responses of alveolar macrophages.
Disease-associated mutations in cytoplasmic loops 1 and 2 of cystic fibrosis transmembrane conductance regulator impede processing or opening of the channel.
Disease-associated mutations in the extracytoplasmic loops of cystic fibrosis transmembrane conductance regulator do not impede biosynthetic processing but impair chloride channel stability.
Disease-associated mutations in the fourth cytoplasmic loop of cystic fibrosis transmembrane conductance regulator compromise biosynthetic processing and chloride channel activity.
Disease-relevant mutations alter amino acid co-evolution networks in the second nucleotide binding domain of CFTR.
Disease-relevant proteostasis regulation of cystic fibrosis transmembrane conductance regulator.
Dispersion of Endoplasmic Reticulum-associated Compartments by 4-phenyl Butyric Acid in Yeast Cells.
Disproportionate head growth retardation in cystic fibrosis.
Disrupted local innervation results in less VIP expression in CF mice tissues.
Disrupted post-transcriptional regulation of the cystic fibrosis transmembrane conductance regulator (CFTR) by a 5'UTR mutation is associated with a CFTR-related disease.
Disrupted tight junctions in the small intestine of cystic fibrosis mice.
Disruption of AtMRP4, a guard cell plasma membrane ABCC-type ABC transporter, leads to deregulation of stomatal opening and increased drought susceptibility.
Disruption of biofilms and killing of Burkholderia cenocepacia from cystic fibrosis lung using an antioxidant-antibiotic combination therapy.
Disruption of CFTR-dependent lipid rafts reduces bacterial levels and corneal disease in a murine model of Pseudomonas aeruginosa keratitis.
Disruption of cytokeratin-8 interaction with F508del-CFTR corrects its functional defect.
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.
Disruption of monolayer integrity enables activation of a cystic fibrosis "bypass" channel in human airway epithelia.
Disruption of the cystic fibrosis transmembrane conductance regulator gene in embryonic stem cells by gene targeting.
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.
Dissection of the Role of VIMP in Endoplasmic Reticulum-Associated Degradation of CFTR?F508.
Distal intestinal obstruction syndrome in adults with cystic fibrosis.
Distinct and specific functions of cGMP-dependent protein kinases.
Distinct Mg(2+)-dependent steps rate limit opening and closing of a single CFTR Cl(-) channel.
Distinct roles for the Hsp40 and Hsp90 molecular chaperones during cystic fibrosis transmembrane conductance regulator degradation in yeast.
Distinct spectrum of CFTR gene mutations in congenital absence of vas deferens.
Distribution and regulation of ENaC subunit and CFTR mRNA expression in murine female reproductive tract.
Distribution of aquaporin water channels AQP1 and AQP5 in the ductal system of the human pancreas.
Distribution of CFTR mutations in Saguenay- Lac-Saint-Jean: proposal of a panel of mutations for population screening.
Distribution of CFTR variations in an Indonesian enteric fever cohort.
Distribution of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mutations in a Cohort of Patients Residing in Palestine.
Distribution patterns of the delta F508 mutation in the CFTR gene of CF-linked marker haplotypes in the German population.
Disturbances of colonic ion secretion in inflammation: role of the enteric nervous system and cAMP.
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.
Divergent CFTR orthologs respond differently to the channel inhibitors CFTRinh-172, glibenclamide, and GlyH-101.
Divergent signaling via SUMO modification: potential for CFTR modulation.
Diverse genotypical features and impacts on clinical course and severity of cystic fibrosis: early childhood experience.
Diversified Synthetic Pathway of 1, 4-Dihydropyridines: A Class of Pharmacologically Important Molecules.
DNA immobilisation procedures for surface plasmon resonance imaging (SPRI) based microarray systems.
DNA Methylation Patterns Correlate with the Expression of SCNN1A, SCNN1B, and SCNN1G (Epithelial Sodium Channel, ENaC) Genes.
DNA polymorphisms in potential regulatory elements of the CFTR gene alter transcription factor binding.
Does cystic fibrosis neonatal screening detect atypical CF forms? Extended genetic characterization and 4-year clinical follow-up.
Does deficiency of arylsulfatase B have a role in cystic fibrosis?
Does epithelial sodium channel hyperactivity contribute to cystic fibrosis lung disease?
Does ivacaftor interfere with the antimicrobial activity of commonly used antibiotics against Pseudomonas aeruginosa?-Results of an in vitro study.
Does stimulation of NaCl secretion in in vitro perfused rectal gland tubules of squalus acanthias increase membrane capacitance?
Does the {Delta}F508-CFTR mutation induce a proinflammatory response in human airway epithelial cells?
Does type 2 diabetes mellitus delay renal failure in autosomal dominant polycystic kidney disease?
Domain interactions in the yeast ATP binding cassette transporter Ycf1p: intragenic suppressor analysis of mutations in the nucleotide binding domains.
Domain interdependence in the biosynthetic assembly of CFTR.
Domain location within the cystic fibrosis transmembrane conductance regulator protein investigated by electron microscopy and gold labelling.
Domain-domain associations in cystic fibrosis transmembrane conductance regulator.
Domain-interface dynamics of CFTR revealed by stabilizing nanobodies.
Double-gradient DGGE for optimized detection of DNA point mutations.
Down regulation of small intestinal ion transport in PDZK1- (CAP70/NHERF3) deficient mice.
Down-regulated CFTR during aging contributes to benign prostatic hyperplasia.
Down-regulation of cystic fibrosis transmembrane conductance regulator gene expression by agents that modulate intracellular divalent cations.
Down-regulation of the anti-inflammatory protein annexin A1 in cystic fibrosis knock-out mice and patients.
Down-regulation of volume-sensitive Cl- channels by CFTR is mediated by the second nucleotide-binding domain.
Downregulation of CFTR Is Involved in the Formation of Hypertrophic Scars.
Downregulation of CFTR promotes epithelial-to-mesenchymal transition and is associated with poor prognosis of breast cancer.
Downregulation of epithelial sodium channel (ENaC) by CFTR co-expressed in Xenopus oocytes is independent of Cl- conductance.
Drug Development for Cystic Fibrosis.
Drug discovery and epithelial physiology.
Drug discovery in academia.
Drug-induced dyspnea versus cystic fibrosis exacerbation: a diagnostic dilemma.
Drug-induced secretory diarrhea: A role for CFTR.
Drug-set enrichment analysis: a novel tool to investigate drug mode of action.
Drugs and their molecular targets: an updated overview.
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.
Dual effects of ADP and adenylylimidodiphosphate on CFTR channel kinetics show binding to two different nucleotide binding sites.
Dual regulation of cardiac Na+-K+ pumps and CFTR Cl- channels by protein kinases A and C.
Dual role of CFTR in cAMP-stimulated HCO3- secretion across murine duodenum.
Dual roles of the sixth transmembrane segment of the CFTR chloride channel in gating and permeation.
Ductular network formation by rat biliary epithelial cells in the dynamical culture with collagen gel and dimethylsulfoxide stimulation.
Duodenal brush border intestinal alkaline phosphatase activity affects bicarbonate secretion in rats.
Duplicated CFTR isoforms in eels diverged in regulatory structures and osmoregulatory functions.
Dynamic activation of cystic fibrosis transmembrane conductance regulator by type 3 and type 4D phosphodiesterase inhibitors.
Dynamic control of cystic fibrosis transmembrane conductance regulator Cl(-)/HCO3(-) selectivity by external Cl(-).
Dynamic Regulation of CFTR Bicarbonate Permeability by [Cl-]i and its Role in Pancreatic Bicarbonate Secretion.
Dynamic regulation of cystic fibrosis transmembrane conductance regulator by competitive interactions of molecular adaptors.
Dynamically regulated CFTR expression and its functional role in cutaneous wound healing.
Dynamics intrinsic to cystic fibrosis transmembrane conductance regulator function and stability.
Dynamics of bacterial colonisation in the respiratory tract of patients with 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.
Dysfunction of CFTR bearing the delta F508 mutation.
Dysfunction of the non-neuronal cholinergic system in the airways and blood cells of patients with cystic fibrosis.
Dysfunctional CFTR alters the bactericidal activity of human macrophages against Pseudomonas aeruginosa.
Dysfunctional cystic fibrosis transmembrane conductance regulator inhibits phagocytosis of apoptotic cells with proinflammatory consequences.
Dysfunctional Inflammation in Cystic Fibrosis Airways: From Mechanisms to Novel Therapeutic Approaches.
Dysregulated signalling pathways in innate immune cells with cystic fibrosis mutations.
Dysregulation of Circadian Rhythm Gene Expression in Cystic Fibrosis Mice.
Dysregulation of proteoglycan production by intrahepatic biliary epithelial cells bearing defective (delta-f508) cystic fibrosis transmembrane conductance regulator.
E3KARP mediates the association of ezrin and protein kinase A with the cystic fibrosis transmembrane conductance regulator in airway cells.
Early activation of sperm by HCO(3)(-) is regulated hormonally in the murine uterus.
Early acute pancreatitis in a child with compound heterozygosis ?F508/R1438W/Y1032C cystic fibrosis: a case report.
Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice.
Early Diagnosis and Intervention in Cystic Fibrosis: Imagining the Unimaginable.
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.
Economic implications of newborn screening for cystic fibrosis: a cost of illness retrospective cohort study.
EDNRA variants associate with smooth muscle mRNA levels, cell proliferation rates, and cystic fibrosis pulmonary disease severity.
Effect of acupuncture on chronic bronchitis: A protocol for systematic review and meta-analysis.
Effect of Alpha-1 Antitrypsin on CFTR Levels in Primary Human Airway Epithelial Cells Grown at the Air-Liquid-Interface.
Effect of anion transport blockers on CFTR in the human sweat duct.
Effect of Annexin A5 on CFTR: regulated traffic or scaffolding?
Effect of ATP concentration on CFTR Cl- channels: a kinetic analysis of channel regulation.
Effect of ATP-sensitive K+ channel regulators on cystic fibrosis transmembrane conductance regulator chloride currents.
Effect of cAMP agonists on cell pH and anion transport by cultured rat inner medullary collecting duct cells.
Effect of cAMP on intracellular and extracellular ATP content of Cl- -secreting epithelia and 3T3 fibroblasts.
Effect of CFTR correctors on the traffic and the function of intracellularly retained ABCB4 variants.
Effect of CFTR modulator therapy on cystic fibrosis-related diabetes.
Effect of CFTR Modulators on Anthropometric Parameters in Individuals with Cystic Fibrosis: An Evidence Analysis Center Systematic Review.
Effect of chloride channel blockers on the cardiac CFTR chloride and L-type calcium currents.
Effect of cystic fibrosis-associated mutations in the fourth intracellular loop of cystic fibrosis transmembrane conductance regulator.
Effect of cytosolic pH on epithelial Na+ channel in normal and cystic fibrosis sweat ducts.
Effect of deleting the R domain on CFTR-generated chloride channels.
Effect of deletion mutations on the function of CFTR chloride channels.
Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study.
Effect of highly effective modulator therapy on quality of life in adults with cystic fibrosis.
Effect of IBMX and alkaline phosphatase inhibitors on Cl- secretion in G551D cystic fibrosis mutant mice.
Effect of inflammatory stimuli on airway ion transport.
Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR.
Effect of ivacaftor therapy on exhaled nitric oxide in patients with cystic fibrosis.
Effect of L-ascorbate on chloride transport in freshly excised sinonasal epithelia.
Effect of lentivirus-mediated CFTR overexpression on oxidative stress injury and inflammatory response in the lung tissue of COPD mouse model.
Effect of phenol red and steroid hormones on cystic fibrosis transmembrane conductance regulator in mouse endometrial epithelial cells.
Effect of salinity and temperature on the expression of genes involved in branchial ion transport processes in European sea bass.
Effect of the E4 region on the persistence of transgene expression from adenovirus vectors.
Effect of the liquorice root derivatives on salt and water balance in a teleost fish, rainbow trout (Oncorhynchus mykiss).
Effect of TongXie-YaoFang on Cl(-) and HCO3 (-) Transport in Diarrhea-Predominant Irritable Bowel Syndrome Rats.
Effect of topiramate on sweat chloride level while screening for cystic fibrosis.
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
Effective silencing of ENaC by siRNA delivered with epithelial-targeted nanocomplexes in human cystic fibrosis cells and in mouse lung.
Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations.
Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial.
Effects and interaction of dietary electrolyte balance and citric acid on the intestinal function of weaned piglets.
Effects of a new cystic fibrosis transmembrane conductance regulator inhibitor on Cl- conductance in human sweat ducts.
Effects of airway surface liquid pH on host defense in cystic fibrosis.
Effects of antiallergic herbal agents on cystic fibrosis transmembrane conductance regulator in nasal mucosal epithelia of allergic rhinitis rabbits.
Effects of Azithromycin (AZM) on Glutathione-S-Transferases (GST)s in Cystic Fibrosis Airway Cells.
Effects of beta-agonists on airway epithelial cells.
Effects of C-terminal deletions on cystic fibrosis transmembrane conductance regulator function in cystic fibrosis airway epithelia.
Effects of carbon monoxide on ion transport across rat distal colon.
Effects of CFTR gene silencing by siRNA or the luminal application of a CFTR activator on fluid secretion from guinea-pig pancreatic duct cells.
Effects of CFTR modulators on pharmacokinetics of tobramycin during acute pulmonary exacerbations in the pediatric cystic fibrosis population.
Effects of chlorothalonil, prochloraz and the combination on intestinal barrier function and glucolipid metabolism in the liver of mice.
Effects of Cordyceps sinensis, Cordyceps militaris and their isolated compounds on ion transport in Calu-3 human airway epithelial cells.
Effects of curcumin on ion channels and transporters.
Effects of cystic fibrosis transmembrane conductance regulator and DeltaF508CFTR on inflammatory response, ER stress, and Ca2+ of airway epithelia.
Effects of early and late pneumothorax drainage on the development of pulmonary oedema.
Effects of indacaterol on the LPS-evoked changes in fluid secretion rate and pH in swine tracheal membrane.
Effects of Long-Term Exercise on Liver Cyst in Polycystic Liver Disease Model Rats.
Effects of lovastatin on trafficking of cystic fibrosis transmembrane conductance regulator in human tracheal epithelium.
Effects of low salinity media on growth, condition, and gill ion transporter expression in juvenile Gulf killifish, Fundulus grandis.
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.
Effects of methacholine and uridine 5'-triphosphate on tracheal mucus rheology in mice.
Effects of mutations in cAMP-dependent protein kinase on chloride efflux in Caco-2 human colonic carcinoma cells.
Effects of nicorandil on the cAMP-dependent Cl- current in guinea-pig ventricular cells.
Effects of P-glycoprotein expression on cyclic AMP and volume-activated ion fluxes and conductances in HT-29 colon adenocarcinoma cells.
Effects of potassium ion supplementation on survival and ion regulation in Gulf killifish Fundulus grandis larvae reared in ion deficient saline waters.
Effects of protein phosphatase and kinase inhibitors on Ca2+ and Cl- currents in guinea pig ventricular myocytes.
Effects of purinergic stimulation, CFTR and osmotic stress on amiloride-sensitive Na+ transport in epithelia and Xenopus oocytes.
Effects of pyrophosphate and nucleotide analogs suggest a role for ATP hydrolysis in cystic fibrosis transmembrane regulator channel gating.
Effects of rhinovirus infection on the expression and function of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel in human nasal mucosa.
Effects of salinity and prolactin on gene transcript levels of ion transporters, ion pumps and prolactin receptors in Mozambique tilapia intestine.
Effects of sevoflurane on the cAMP-induced short-circuit current in mouse tracheal epithelium and recombinant Cl- (CFTR) and K+ (KCNQ1) channels.
Effects of Slc26a6 deletion and CFTR inhibition on HCO3- secretion by mouse pancreatic duct.
Effects of the delta F508 mutation on the structure, function, and folding of the first nucleotide-binding domain of CFTR.
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.
Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation.
Efficacy and Safety of the CFTR Potentiator Icenticaftor (QBW251) in COPD: Results from a Phase 2 Randomized Trial.
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.
Efficacy of lumacaftor-ivacaftor for the treatment of cystic fibrosis patients homozygous for the F508del-CFTR mutation.
Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis.
Efficiency of plasmid delivery and expression after lipid-mediated gene transfer to human cells in vitro.
Efficient 12-mutation testing in the CFTR gene: a general model for complex mutation analysis.
Efficient Activation of Pathogenic ?Phe501 Mutation in Monocarboxylate Transporter 8 by Chemical and Pharmacological Chaperones.
Efficient approximations for learning phylogenetic HMM models from data.
Efficient endocytosis of the cystic fibrosis transmembrane conductance regulator requires a tyrosine-based signal.
Efficient Gene Editing at Major CFTR Mutation Loci.
Efficient gene transfer into myocardium by direct injection of adenovirus vectors.
Efficient intracellular processing of the endogenous cystic fibrosis transmembrane conductance regulator in epithelial cell lines.
Efficient intratracheal delivery of airway epithelial cells in mice and pigs.
Efficient killing of inhaled bacteria in DeltaF508 mice: role of airway surface liquid composition.
EG-VEGF, BV8, and their receptor expression in human bronchi and their modification in cystic fibrosis: Impact of CFTR mutation (delF508).
Electrochemical immunosensors for the detection of survival motor neuron (SMN) protein using different carbon nanomaterials-modified electrodes.
Electrodiffusional ATP movement through CFTR and other ABC transporters.
Electrodiffusional ATP movement through the cystic fibrosis transmembrane conductance regulator.
Electrogenic bicarbonate secretion by prairie dog gallbladder.
Electrogenic Na?/HCO?? co-transporter-1 is essential for the parathyroid hormone-stimulated intestinal HCO?? secretion.
Electrolyte transport in the mammalian colon: mechanisms and implications for disease.
Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host defense.
Electronic health record phenotypes associated with genetically regulated expression of CFTR and application to cystic fibrosis.
Electrophysiological Approaches for the Study of Ion Channel Function.
Electrophysiological characterization of chloride secretion across the jejunum and colon of pigs as affected by age and weaning.
Electrophysiological evidence for the presence of cystic fibrosis transmembrane conductance regulator (CFTR) in mouse sperm.
Electrostatic Tuning of Anion Attraction from the Cytoplasm to the Pore of the CFTR Chloride Channel.
Electrostatic tuning of the pre- and post-hydrolytic open states in CFTR.
Elevated hepatic SULT1E1 activity in mouse models of cystic fibrosis alters the regulation of estrogen responsive proteins.
Elevated paracellular glucose flux across cystic fibrosis airway epithelial monolayers is an important factor for Pseudomonas aeruginosa growth.
Elevated tear fluid levels of MIP-1alpha in patients with cystic fibrosis.
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Elexacaftor-Tezacaftor-Ivacaftor: The First Triple-Combination Cystic Fibrosis Transmembrane Conductance Regulator Modulating Therapy.
Elexacaftor-tezacaftor-ivacaftor: The new paradigm to treat people with cystic fibrosis with at least one p.Phe508del mutation.
Elexacaftor/Ivacaftor/Tezacaftor: First Approval.
Embryology of the Absent Vas Supported by 2 Cases of Congenital Unilateral Absence of Vas With Varied Associations.
Embryonic epithelial membrane transporters.
Embryonic lung growth is normal in a cftr-knockout mouse model.
Emerging Cystic Fibrosis Transmembrane Conductance Regulator Modulators as New Drugs for Cystic Fibrosis: A Portrait of in Vitro Pharmacology and Clinical Translation.
Emerging drug treatments for cystic fibrosis.
Emerging drugs for cystic fibrosis.
Emerging microRNA Therapeutic Approaches for Cystic Fibrosis.
Emerging new paradigms for ABCG transporters.
Emerging pharmacologic therapies for irritable bowel syndrome.
Emerging role of AMP-activated protein kinase in coupling membrane transport to cellular metabolism.
Emerging role of CFTR as an epigenetic regulator - linking environmental cues to microRNAs.
Emerging role of cystic fibrosis transmembrane conductance regulator - an epithelial chloride channel in gastrointestinal cancers.
Emerging Technologies for Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Restoration in all People with CF.
Emerging therapies for cystic fibrosis lung disease.
Emerging therapies in cystic fibrosis.
ENaC inhibition in cystic fibrosis: potential role in the new era of CFTR modulator therapies.
ENaC regulation by phospholipids and DGK explained through mathematical modeling.
ENaC- and CFTR-dependent ion and fluid transport in human middle ear epithelial cells.
ENaC- and CFTR-dependent ion and fluid transport in mammary epithelia.
ENaC-mediated sodium influx exacerbates NLRP3-dependent inflammation in Cystic Fibrosis.
Enamel mineral composition of normal and cystic fibrosis transgenic mice.
Enamel pathology resulting from loss of function in the cystic fibrosis transmembrane conductance regulator in a porcine animal model.
Endocervical metaplasia of the endometrium in a patient with cystic fibrosis: a case report.
Endocytic adaptor complexes bind the C-terminal domain of CFTR.
Endocytic trafficking of CFTR in health and disease.
Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator.
Endogenous ATP release inhibits electrogenic Na(+) absorption and stimulates Cl (-) secretion in MDCK cells.
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.
Endogenous expression of type II cGMP-dependent protein kinase mRNA and protein in rat intestine. Implications for cystic fibrosis transmembrane conductance regulator.
Endogenous luminal surface adenosine signaling regulates duodenal bicarbonate secretion in rats.
Endogenous or overexpressed cGMP-dependent protein kinases inhibit cAMP-dependent renin release from rat isolated perfused kidney, microdissected glomeruli, and isolated juxtaglomerular cells.
Endogenous surface expression of ?F508-CFTR mediates cAMP-stimulated Cl(-) current in CFTR(?F508/?F508) pig thyroid epithelial cells.
Endoplasmic reticulum stress and the unfolded protein response regulate genomic cystic fibrosis transmembrane conductance regulator expression.
Endosomal SNARE proteins regulate CFTR activity and trafficking in epithelial cells.
Energy Balance and Mechanisms of Weight Gain with Ivacaftor Treatment of Cystic Fibrosis Gating Mutations.
Engineered transfer RNAs for suppression of premature termination codons.
Engraftment of donor-derived epithelial cells in multiple organs following bone marrow transplantation into newborn mice.
Enhanced cell-surface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones.
Enhanced delivery of peptide-morpholino oligonucleotides with a small molecule to correct splicing defects in the lung.
Enhanced expression of ncc1 and clc2c in the kidney and urinary bladder accompanies freshwater acclimation in Mozambique tilapia.
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.
ENHANCEMENT OF ALVEOLAR EPITHELIAL SODIUM CHANNEL ACTIVITY WITH DECREASED CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR EXPRESSION IN MOUSE LUNG.
Enhancement of ciliary beat amplitude by carbocisteine in ciliated human nasal epithelial cells.
Enhancement of liposome-mediated gene transfer to human airway epithelial cells by replication-deficient adenovirus.
Enhancing Cystic Fibrosis Immune Regulation.
Enhancing the efficiency of introducing precise mutations into the mouse genome by hit and run gene targeting.
Enhancing the Potency of F508del Correction: A Multi-Layer Combinational Approach to Drug Discovery for Cystic Fibrosis.
Enteric nervous system: sensory physiology, diarrhea and constipation.
Enteric oxalate secretion is not directly mediated by the human CFTR chloride channel.
Enterohemorrhagic E. coli (EHEC)-Secreted Serine Protease EspP Stimulates Electrogenic Ion Transport in Human Colonoid Monolayers.
Enteroinvasive bacteria alter barrier and transport properties of human intestinal epithelium: role of iNOS and COX-2.
Enzymatic and chemical probing of an S1 nuclease-sensitive site upstream from the human CFTR gene.
EPAC1 activation by cAMP stabilizes CFTR at the membrane by promoting its interaction with NHERF1.
Epidemiology and a novel procedure for large scale analysis of CFTR rearrangements in classic and atypical CF patients: a multicentric Italian study.
Epidemiology and genetics of cystic fibrosis in Asia: In preparation for the next-generation treatments.
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.
Epigenetic Modification of CFTR in Head and Neck Cancer.
Epigenetic regulation of CFTR in salivary gland.
Epinephrine Stimulation of Anion Secretion in the Calu-3 Serous Cell Model.
Episomal expression of wild-type CFTR corrects cAMP-dependent chloride transport in respiratory epithelial cells.
Epithelial Barrier Properties of the 16HBE14o- Human Bronchial Epithelial Cell Culture Model.
Epithelial cell contact-induced alterations in Salmonella enterica serovar Typhi lipopolysaccharide are critical for bacterial internalization.
Epithelial Chloride Transport by CFTR Requires TMEM16A.
Epithelial IgG and its relationship to the loss of F508 in the common mutant form of the cystic fibrosis transmembrane conductance regulator.
Epithelial ion channels in the regulation of female reproductive tract fluid microenvironment: implications in fertility and infertility.
Epithelial mesenchymal transition (EMT): a universal process in lung diseases with implications for cystic fibrosis pathophysiology.
Epithelial Na(+) channel inhibitors for the treatment of cystic fibrosis.
Epithelial sodium channel inhibition in primary human bronchial epithelia by transfected siRNA.
Epithelial sodium channel silencing as a strategy to correct the airway surface fluid deficit in cystic fibrosis.
Epithelial sodium channels (ENaC) are uniformly distributed on motile cilia in the oviduct and the respiratory airways.
Epithelial sodium channels regulate cystic fibrosis transmembrane conductance regulator chloride channels in Xenopus oocytes.
Epithelial transport in polycystic kidney disease.
Epitope conservation and immunohistochemical localization of the guanylin/stable toxin peptide receptor, guanylyl cyclase C.
Epitope tagging permits cell surface detection of functional CFTR.
Epoxide-Mediated CifR Repression of cif Gene Expression Utilizes Two Binding Sites in Pseudomonas aeruginosa.
ER-associated complexes (ERACs) containing aggregated cystic fibrosis transmembrane conductance regulator (CFTR) are degraded by autophagy.
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.
ERp29 Regulates Epithelial Sodium Channel Functional Expression by Promoting Channel Cleavage.
Erratum to: A Stable Human-Cell System Overexpressing Cystic Fibrosis Transmembrane Conductance Regulator Recombinant Protein at the Cell Surface.
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.
Escherichia coli-induced epithelial hyporesponsiveness to secretagogues is associated with altered CFTR localization.
Essential fatty acid deficiency in relation to genotype in patients with cystic fibrosis.
Establishing a diagnosis of cystic fibrosis.
Establishment and characterization of a novel polarized MDCK epithelial cellular model for CFTR studies.
Establishment and long-term culture of human cystic fibrosis endothelial cells.
Estimating the age of p.(Phe508del) with family studies of geographically distinct European populations and the early spread of 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.
Estrogen and the cystic fibrosis gender gap.
Estrogen regulation of ion transporter messenger RNA levels in mouse efferent ductules are mediated differentially through estrogen receptor (ER) alpha and ER beta.
Estrogen upregulates the expression levels and functional activities of duodenal mucosal CFTR and SLC26A6.
Estrogen-dependent expression of the cystic fibrosis transmembrane regulator gene in a novel uterine epithelial cell line.
Estrogen-induced abnormally high cystic fibrosis transmembrane conductance regulator expression results in ovarian hyperstimulation syndrome.
Ethanol and its non-oxidative metabolites profoundly inhibit CFTR function in pancreatic epithelial cells which is prevented by ATP supplementation.
Ethical dilemma: ELX/TEZ/IVA or Lung Transplantation in Cystic Fibrosis and End Stage Lung Disease?
Ethnic heterogeneity and cystic fibrosis transmembrane regulator (CFTR) mutation frequencies in Chicago-area CF families.
Etiology of azoospermia in 100 consecutive nonvasectomized men.
European Academy of Andrology guideline Management of oligo-astheno-teratozoospermia.
Evaluating sequence data quality from the Swift Accel-Amplicon CFTR Panel.
Evaluation in vitro and in vivo of cationic liposome-expression construct complexes for cystic fibrosis gene therapy.
Evaluation of 1,2,3-Triazoles as Amide Bioisosteres In Cystic Fibrosis Transmembrane Conductance Regulator Modulators VX-770 and VX-809.
Evaluation of a BeadXpress Assay for a 151-Mutation and Variant CFTR Screening Panel After 11,000 Samples: Implications for Practice.
Evaluation of autophagy inducers in epithelial cells carrying the ?F508 mutation of the cystic fibrosis transmembrane conductance regulator CFTR.
Evaluation of gene targeting by homologous recombination in ovine somatic cells.
Evaluation of high-resolution melting (HRM) for mutation scanning of selected exons of the CFTR gene.
Evaluation of MRP1-5 gene expression in cystic fibrosis patients homozygous for the delta F508 mutation.
Evaluation of potential regulatory elements identified as DNase I hypersensitive sites in the CFTR gene.
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.
Evaluation of the efficacy and safety of in vitro, adenovirus-mediated transfer of the human cystic fibrosis transmembrane conductance regulator cDNA.
Evidence against defective trans-Golgi acidification in cystic fibrosis.
Evidence against the acidification hypothesis in cystic fibrosis.
Evidence for a role of arginine vasotocin receptors in the gill during salinity acclimation by a euryhaline teleost fish.
Evidence for alteration of calpain/calpastatin system in PBMC of cystic fibrosis patients.
Evidence for an apical Na-Cl cotransporter involved in ion uptake in a teleost fish.
Evidence for cystic fibrosis transmembrane conductance regulator chloride current in swine ventricular myocytes.
Evidence for direct interaction between actin and the cystic fibrosis transmembrane conductance regulator.
Evidence for functional atypical nicotinic receptors that activate K+ dependent Cl- secretion in mouse tracheal epithelium.
Evidence for independent Cl- and HCO3- secretion and involvement of an apical Na(+)-HCO3- cotransporter in cultured rat epididymal epithelia.
Evidence for location of the CFTR in human placental apical membrane vesicles.
Evidence for phosphorylation of serine 753 in CFTR using a novel metal-ion affinity resin and matrix-assisted laser desorption mass spectrometry.
Evidence for safety and efficacy of DOTAP cationic liposome mediated CFTR gene transfer to the nasal epithelium of patients with cystic fibrosis.
Evidence for the Involvement of Lipid Rafts and Plasma Membrane Sphingolipid Hydrolases in
Evidence of a functional CFTR Cl(-) channel in adult alveolar epithelial cells.
Evidence of Absorptive Function in vivo in a Neo-Formed Bio-Artificial Intestinal Segment Using a Rodent Model.
Evidence that Calu-3 human airway cells secrete bicarbonate.
Evidence that endoplasmic reticulum (ER)-associated degradation of cystic fibrosis transmembrane conductance regulator is linked to retrograde translocation from the ER membrane.
Evidence that extracellular anions interact with a site outside the CFTR chloride channel pore to modify channel properties.
Evidence that hereditary pancreatitis is genetically heterogeneous disorder.
Evidence that release of adenosine triphosphate from endothelial cells during increased shear stress is vesicular.
Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis.
Evolutionary and functional divergence between the cystic fibrosis transmembrane conductance regulator and related ATP-binding cassette transporters.
Evolving Mechanistic Views and Emerging Therapeutic Strategies for Cystic Fibrosis-Related Diabetes.
Ex vivo biochemical analysis of CFTR in human rectal biopsies.
Examination of potential overlap in autism and language loci on chromosomes 2, 7, and 13 in two independent samples ascertained for specific language impairment.
Examining basal chloride transport using the nasal potential difference response in a murine model.
Excess fluoride interferes with chloride-channel-dependent endocytosis in ameloblasts.
Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa.
Exocytosis is not involved in activation of Cl- secretion via CFTR in Calu-3 airway epithelial cells.
Exome sequencing of Saudi Arabian patients with ADPKD.
Exon 9 of the CFTR gene: splice site haplotypes and cystic fibrosis mutations.
Exon skipping through the creation of a putative exonic splicing silencer as a consequence of the cystic fibrosis mutation R553X.
Exophiala dermatitidis Revealing Cystic Fibrosis in Adult Patients with Chronic Pulmonary Disease.
Experimental assessment of splicing variants using expression minigenes and comparison with in silico predictions.
Exploiting species differences to understand the CFTR Cl- channel.
Exploring the mechanisms of macrolides in cystic fibrosis.
Exposure of Pseudomonas aeruginosa to bactericidal hypochlorous acid during neutrophil phagocytosis is compromised in cystic fibrosis.
Exposure to cigarette smoke condensate reduces calcium activated chloride channel transport in primary sinonasal epithelial cultures.
Exposure to sodium butyrate leads to functional downregulation of calcium-activated potassium channels in human airway epithelial cells.
Expression and characterization of the cystic fibrosis transmembrane conductance regulator.
Expression and chromosome localization of the murine cystic fibrosis transmembrane conductance regulator.
Expression and degradation of the cystic fibrosis transmembrane conductance regulator in Saccharomyces cerevisiae.
Expression and Distribution of Cystic Fibrosis Transmembrane Conductance Regulator in Neurons of the Human Brain.
Expression and distribution of cystic fibrosis transmembrane conductance regulator in neurons of the spinal cord.
Expression and distribution of the Na(+)-HCO(-)(3) cotransporter in human pancreas.
Expression and function of Anoctamin 1/TMEM16A calcium-activated chloride channels in airways of in vivo mouse models for cystic fibrosis research.
Expression and function of CLC and cystic fibrosis transmembrane conductance regulator chloride channels in renal epithelial tubule cells: pathophysiological implications.
Expression and function of cystic fibrosis transmembrane conductance regulator in rat intrapulmonary arteries.
Expression and functional characterization of CFTR in mast cells.
Expression and functional properties of the second predicted nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator fused to glutathione-S-transferase.
Expression and intracellular processing of chimeric and mutant CFTR molecules.
Expression and localization of cystic fibrosis transmembrane conductance regulator in human gingiva.
Expression and localization of cystic fibrosis transmembrane conductance regulator in the rat endocrine pancreas.
Expression and localization of the cystic fibrosis transmembrane conductance regulator mRNA and its protein in rat brain.
Expression and purification of the cystic fibrosis transmembrane conductance regulator protein in Saccharomyces cerevisiae.
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.
Expression and purification of two hydrophobic double-spanning membrane proteins derived from the cystic fibrosis transmembrane conductance regulator.
Expression and regulation of chloride channels in neonatal rat cardiomyocytes.
Expression and regulation of the cystic fibrosis gene during rat liver regeneration.
Expression and regulation of the Na+-K+-2Cl- cotransporter NKCC1 in the normal and CFTR-deficient murine colon.
Expression and Role of CFTR in Human Esophageal Squamous Cell Carcinoma.
Expression in Escherichia coli of cytoplasmic portions of the cystic fibrosis transmembrane conductance regulator: apparent bacterial toxicity of peptides containing R-domain sequences.
Expression of a truncated cystic fibrosis transmembrane conductance regulator with an AAV5-pseudotyped vector in primates.
Expression of alpha v beta 5 integrin is necessary for efficient adenovirus-mediated gene transfer in the human airway.
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.
Expression of an ATP-binding cassette transporter-encoding gene (YOR1) is required for oligomycin resistance in Saccharomyces cerevisiae.
Expression of carbonic anhydrase, cystic fibrosis transmembrane regulator (CFTR) and V-H(+)-ATPase in the lancelet Branchiostoma lanceolatum (Pallas, 1774).
Expression of CFTR and a cAMP-stimulated chloride secretory current in cultured human fetal alveolar epithelial cells.
Expression of CFTR and Cl(-) conductances in cells of pulmonary neuroepithelial bodies.
Expression of CFTR and presence of cAMP-mediated fluid secretion in human fetal lung.
Expression of CFTR controls cAMP-dependent activation of epithelial K+ currents.
Expression of CFTR from a ciliated cell-specific promoter is ineffective at correcting nasal potential difference in CF mice.
Expression of CFTR in human and bovine thyroid epithelium.
Expression of Cl- channels/transporters in nasal polyps.
Expression of cystic fibrosis transmembrane conductance regulator alters the responses to hypotonic cell swelling and ATP of Chinese hamster ovary cells.
Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells.
Expression of cystic fibrosis transmembrane conductance regulator during early human embryo development.
Expression of cystic fibrosis transmembrane conductance regulator in a model epithelium.
Expression of Cystic Fibrosis Transmembrane Conductance Regulator in Ganglia of Human Gastrointestinal Tract.
Expression of cystic fibrosis transmembrane conductance regulator in ganglion cells of the hearts.
Expression of cystic fibrosis transmembrane conductance regulator in human endometrium.
Expression of cystic fibrosis transmembrane conductance regulator in human gallbladder epithelial cells.
Expression of cystic fibrosis transmembrane conductance regulator in liver tissue from patients with cystic fibrosis.
Expression of cystic fibrosis transmembrane conductance regulator in paracervical ganglia.
Expression of cystic fibrosis transmembrane conductance regulator in rat efferent duct epithelium.
Expression of cystic fibrosis transmembrane conductance regulator in rat ovary.
Expression of cystic fibrosis transmembrane conductance regulator in the human distal lung.
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.
Expression of cystic fibrosis transmembrane regulator Cl- channels in heart.
Expression of delta F508 cystic fibrosis transmembrane conductance regulator protein and related chloride transport properties in the gallbladder epithelium from cystic fibrosis patients.
Expression of DeltaF508 CFTR in normal mouse lung after site-specific modification of CFTR sequences by SFHR.
Expression of DeltaF508 Cystic Fibrosis Transmembrane Regulator (CFTR) Decreases Membrane Sialylation.
Expression of Drosophila melanogaster P-glycoproteins is associated with ATP channel activity.
Expression of ENaC subunits, chloride channels, and aquaporins in ovine fetal lung: ontogeny of expression and effects of altered fetal cortisol concentrations.
Expression of ion transport-associated proteins in human efferent and epididymal ducts.
Expression of miR?542?3p in osteosarcoma with miRNA microarray data, and its potential signaling pathways.
Expression of S100A8 correlates with inflammatory lung disease in congenic mice deficient of the cystic fibrosis transmembrane conductance regulator.
Expression of SLC26A3, CFTR and NHE3 in the human male reproductive tract: role in male subfertility caused by congenital chloride diarrhoea.
Expression of the chloride channel ClC-2 in the murine small intestine epithelium.
Expression of the chloride channel CLC-K in human airway epithelial cells.
Expression of the cystic fibrosis gene and the major pancreatic mucin gene, MUC1, in human ductal epithelial cells.
Expression of the cystic fibrosis transmembrane conductance regulator (CFTR) mRNA in normal and pathological adult human epididymis.
Expression of the cystic fibrosis transmembrane conductance regulator from a novel adeno-associated virus promoter.
Expression of the cystic fibrosis transmembrane conductance regulator gene can be regulated by protein kinase C.
Expression of the cystic fibrosis transmembrane conductance regulator gene in cells of non-epithelial origin.
Expression of the cystic fibrosis transmembrane conductance regulator gene in the respiratory tract of normal individuals and individuals with cystic fibrosis.
Expression of the cystic fibrosis transmembrane conductance regulator in rat spermatids: implication for the site of action of antispermatogenic agents.
Expression of the human cystic fibrosis transmembrane conductance regulator gene in the mouse lung after in vivo intratracheal plasmid-mediated gene transfer.
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.
Expression of voltage-gated potassium channels in human and mouse colonic carcinoma.
Expression, localization, and binding activity of the ezrin/radixin/moesin proteins in the mouse testis.
Expression, localization, and functional evaluation of CFTR in bovine corneal endothelial cells.
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.
Extensive molecular analysis of patients bearing CFTR-related disorders.
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.
Extensive sequencing of the cystic fibrosis transmembrane regulator gene: assay validation and unexpected benefits of developing a comprehensive test.
Extent of the selectivity filter conferred by the sixth transmembrane region in the CFTR chloride channel pore.
External and Genetic Conditions Determining Male Infertility.
External ATP and its analogs activate the cystic fibrosis transmembrane conductance regulator by a cyclic AMP-independent mechanism.
External Zn(2+) binding to cysteine-substituted cystic fibrosis transmembrane conductance regulator constructs regulates channel gating and curcumin potentiation.
Extracellular phosphate enhances the function of F508del-CFTR rescued by CFTR correctors.
Extracellular UTP stimulates electrogenic bicarbonate secretion across CFTR knockout gallbladder epithelium.
Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry.
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.
Ezrin controls the macromolecular complexes formed between an adapter protein Na+/H+ exchanger regulatory factor and the cystic fibrosis transmembrane conductance regulator.
Ezrin links CFTR to TLR4 signaling to orchestrate anti-bacterial immune response in macrophages.
Ezrin-radixin-moesin-binding phosphoprotein (EBP50), an estrogen-inducible scaffold protein, contributes to biliary epithelial cell proliferation.
F508del CFTR gene mutation in patients with allergic bronchopulmonary aspergillosis.
F508del disturbs the dynamics of the nucleotide binding domains of CFTR before and after ATP hydrolysis.
F508del-CFTR Rescue: A Matter of Cell Stress Response.
Factors influencing readthrough therapy for frequent cystic fibrosis premature termination codons.
Factors influencing the acquisition and eradication of early Pseudomonas aeruginosa infection in cystic fibrosis.
Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers.
Failure of the cystic fibrosis transmembrane conductance regulator to conduct ATP.
False negative newborn screen and neonatal cholestasis in a premature child with cystic fibrosis.
Familial concordance of phenotype and microbial variation among siblings with CF.
Fas and Fas ligand expression in cystic fibrosis airway epithelium.
Fas Expression in Conjunctival Epithelial Cells of Patients With Cystic Fibrosis.
Fatty acid metabolism in cystic fibrosis.
Feline immunodeficiency virus vectors persistently transduce nondividing airway epithelia and correct the cystic fibrosis defect.
Female cystic fibrosis mutation carriers and assisted reproductive technology: does carrier status affect reproductive outcomes?
Female gender hormones regulate mRNA levels and function of the rat lung epithelial Na channel.
Female reproductive health in cystic fibrosis.
Fenretinide differentially modulates the levels of long- and very long-chain ceramides by downregulating Cers5 enzyme: evidence from bench to bedside.
Fenretinide prevents the development of osteoporosis in Cftr-KO mice.
Fertility, Pregnancy and Lactation Considerations for Women with CF in the CFTR Modulator Era.
Fetal Lung Epithelial Ion Channels Relocate in the Cell Membrane During Late Gestation.
FFA3 Activation Stimulates Duodenal Bicarbonate Secretion and Prevents NSAID-Induced Enteropathy via the GLP-2 Pathway in Rats.
First cloning and functional characterization of a melatonin receptor in fish brain: a novel one?
First experience in Switzerland in Phe508del homozygous cystic fibrosis patients with end-stage pulmonary disease enrolled in a lumacaftor-ivacaftor therapy trial - preliminary results.
First report of c. 1499G>C mutation in a 6-month-child with cystic fibrosis.
First report of cystic fibrosis mutations in Libyan cystic fibrosis patients.
First report of the cystic fibrosis transmembrane conductance regulator mutation c.1521_1523delCTT (p. Phe508del) in two Qatari patients with cystic fibrosis.
Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis.
FK506 binding protein 8 peptidylprolyl isomerase activity manages a late stage of cystic fibrosis transmembrane conductance regulator (CFTR) folding and stability.
FKBP38 peptidylprolyl isomerase promotes the folding of cystic fibrosis transmembrane conductance regulator in the endoplasmic reticulum.
FLAG epitope positioned in an external loop preserves normal biophysical properties of CFTR.
Flagellin of Pseudomonas aeruginosa inhibits Na+ transport in airway epithelia.
Flagellin-stimulated Cl- secretion and innate immune responses in airway epithelia: role for p38.
Flickery block of single CFTR chloride channels by intracellular anions and osmolytes.
Flos Magnoliae Inhibits Chloride Secretion via ANO1 Inhibition in Calu-3 Cells.
Flow cytometry analysis reveals a decrease in intracellular sodium during sperm capacitation.
Fluid and electrolyte transport in the small intestine.
Fluid secretion by submucosal glands of the tracheobronchial airways.
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.
Fluorescence assay for simultaneous quantification of CFTR ion-channel function and plasma membrane proximity.
Fluorescence energy transfer detection as a homogeneous DNA diagnostic method.
Fluorescence in situ hybridization mapping of the cystic fibrosis transmembrane conductance regulator (CFTR) gene to 7q31.3.
Fluorescence-based oligonucleotide ligation assay for analysis of cystic fibrosis transmembrane conductance regulator gene mutations.
Fluorescence-based, multiplex allele-specific PCR (MASPCR) detection of the delta F508 deletion in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Fluorescent chloride indicators to assess the efficacy of CFTR cDNA delivery.
Fluorescent multiplex microsatellites used to identify haplotype associations with 15 CFTR mutations in 124 Northern Irish CF families.
Fluoride stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.
Fluoxetine inhibits calcium-activated currents of salamander rod photoreceptor somata and presynaptic terminals via modulation of intracellular calcium dynamics.
Focal adhesion kinase and osmotic responses in ionocytes of Fundulus heteroclitus, a euryhaline teleost fish.
Folate Protects Hepatocytes of Hyperhomocysteinemia Mice From Apoptosis via Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Activated Endoplasmic Reticulum Stress.
Folding and rescue of a cystic fibrosis transmembrane conductance regulator trafficking mutant identified using human-murine chimeric proteins.
Folding of CFTR is predominantly cotranslational.
Fooling a freshwater fish: how dietary salt transforms the rainbow trout gill into a seawater gill phenotype.
Forskolin and PMA pretreatment of HT29 cells alters their chloride conductance induced by cAMP, Ca2+ and hypotonic cell swelling.
Forskolin-induced apical membrane insertion of virally expressed, epitope-tagged CFTR in polarized MDCK cells.
Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients.
Four adult patients with the missense mutation L206W and a mild cystic fibrosis phenotype.
Four case reports of Chinese cystic fibrosis patients and literature review.
Four new mutations of the CFTR gene (541delC, R347H, R352Q, E585X) detected by DGGE analysis in Italian CF patients, associated with different clinical phenotypes.
Freedom of expression. Focus on "Endoplasmic reticulum stress and the unfolded protein response regulate cystic fibrosis transmembrane conductance regulator expression".
Frequencies of cystic fibrosis mutations in the Maine population: high proportion of unknown alleles in individuals of French-Canadian ancestry.
Frequency and clinical significance of the S1235R mutation in the cystic fibrosis transmembrane conductance regulator gene: results from a collaborative study.
Frequency of CFTR gene mutations in males participating in an ICSI programme.
Frequency of CFTR, SPINK1, and Cathepsin B Gene Mutation in North Indian Population: Connections between Genetics and Clinical Data.
Frequency of cystic fibrosis transmembrane conductance regulator gene mutations and 5T allele in patients with allergic bronchopulmonary aspergillosis.
Frequency of Cystic Fibrosis Transmembrane Conductance Regulator Variants in Individuals Evaluated for Primary Ciliary Dyskinesia.
Frequency of delta F508 and haplotype association in Austrian cystic fibrosis families.
Frequency of large CFTR gene rearrangements in Italian CF patients.
Frequency of the delta F508 mutation and flanking marker haplotypes at the CF locus from 167 Czech families.
Frequency of the F508 deletion in the CFTR gene in Turkish cystic fibrosis patients.
Frequent occurrence of the CFTR intron 8 (TG)n 5T allele in men with congenital bilateral absence of the vas deferens.
From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.
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.
Function and expression of cystic fibrosis transmembrane conductance regulator after small intestinal transplantation in mice.
Function and regulation of TRPM7, as well as intracellular magnesium content, are altered in cells expressing ?F508-CFTR and G551D-CFTR.
Function of the R domain in the cystic fibrosis transmembrane conductance regulator chloride channel.
Function of the rat calcitonin receptors, C1a and C1b, expressed in Xenopus oocytes.
Function of the second nucleotide-binding fold in the CFTR chloride channel.
Function of Xenopus cystic fibrosis transmembrane conductance regulator (CFTR) Cl channels and use of human-Xenopus chimeras to investigate the pore properties of CFTR.
Function, evolution and structure of multidrug resistance protein (MRP).
Functional abnormalities in the retinal pigment epithelium of CFTR mutant mice.
Functional Activity of Pat-1 (Slc26a6) Cl(-) /HCO(3) (-) Exchange in the Lower Villus Epithelium of Murine Duodenum.
Functional activity of the CFTR Cl- channel in human myocardium.
Functional analysis of a mammalian odorant receptor subfamily.
Functional analysis of a vacuolar ABC transporter in wild-type Candida albicans reveals its involvement in virulence.
Functional analysis of cis-acting elements regulating the alternative splicing of human CFTR exon 9.
Functional analysis of mutations in the putative binding site for cystic fibrosis transmembrane conductance regulator potentiators. Interaction between activation and inhibition.
Functional analysis of nonsynonymous single nucleotide polymorphisms in human SLC26A9.
Functional analysis of synonymous substitutions predicted to affect splicing of the CFTR gene.
Functional analysis of the C-terminal boundary of the second nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator and structural implications.
Functional Anatomic Imaging of the Airway Surface.
Functional and metabolic impairment in cigarette smoke-exposed macrophages is tied to oxidative stress.
Functional and pharmacological induced structural changes of the cystic fibrosis transmembrane conductance regulator in the membrane solved using SAXS.
Functional architecture of the CFTR chloride channel.
Functional Architecture of the Cytoplasmic Entrance to the Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Pore.
Functional arrangement of the 12th transmembrane region in the CFTR chloride channel pore based on functional investigation of a cysteine-less CFTR variant.
Functional CFTR Expression in Cystic Fibrosis Airway Epithelial Cells by AAV6.2-mediated Segmental Trans-splicing.
Functional Cftr in Crypt Epithelium of Organotypic Enteroid Cultures from Murine Small Intestine.
Functional CFTR in endosomal compartment of CFTR-expressing fibroblasts and T84 cells.
Functional characteristics of L1156F-CFTR associated with alcoholic chronic pancreatitis in Japanese.
Functional characterization of a novel CFTR mutation P67S identified in a patient with atypical cystic fibrosis.
Functional characterization of a recombinant adeno-associated virus 5-pseudotyped cystic fibrosis transmembrane conductance regulator vector.
Functional characterization of cystic fibrosis transmembrane conductance regulator (CFTR) in apical membranes purified from bovine tracheal epithelium.
Functional characterization of various channel-expressing central airway epithelial cells from mouse induced pluripotent stem cells.
Functional characterization reveals that zebrafish CFTR prefers to occupy closed channel conformations.
Functional classification of gill ionocytes and spatiotemporal changes in their distribution after transfer from seawater to freshwater in Japanese seabass.
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.
Functional comparison of mouse slc26a6 anion exchanger with human SLC26A6 polypeptide variants: differences in anion selectivity, regulation, and electrogenicity.
Functional consequences of heterologous expression of the cystic fibrosis transmembrane conductance regulator in fibroblasts.
Functional coupling of Gs and CFTR is independent of their association with lipid rafts in epithelial cells.
Functional cystic fibrosis transmembrane conductance regulator expression in cystic fibrosis airway epithelial cells by AAV6.2-mediated segmental trans-splicing.
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.
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).
Functional Differences in Pore Properties Between Wild-Type and Cysteine-Less Forms of the CFTR Chloride Channel.
Functional dissection of the R domain of cystic fibrosis transmembrane conductance regulator.
Functional domain analysis of the yeast ABC transporter Ycf1p by site-directed mutagenesis.
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.
Functional expression and apical localization of the cystic fibrosis transmembrane conductance regulator in MDCK I cells.
Functional Expression of a Ca(2+)-activated Cl(-) Channel Modulator Involved in Ion Transport and Epithelial Cell Differentiation.
Functional expression of adrenergic and opioid receptors in Xenopus oocytes: interaction between alpha 2- and beta 2-adrenergic receptors.
FUNCTIONAL EXPRESSION OF CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR IN MOUSE CHONDROCYTES.
Functional expression of cystic fibrosis transmembrane conductance regulator in rat oviduct epithelium.
Functional expression of G protein-coupled receptor 30 in immature rat epididymal epithelium.
Functional expression of the cystic fibrosis transmembrane conductance regulator in yeast.
Functional Gene Correction for Cystic Fibrosis in Lung Epithelial Cells Generated from Patient iPSCs.
Functional genomic responses to cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR(delta508) in the lung.
Functional human CFTR produced by stable Chinese hamster ovary cell lines derived using yeast artificial chromosomes.
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.
Functional integrity of the vesicle transporting machinery is required for complete activation of cFTR expressed in xenopus laevis oocytes.
Functional interaction between CFTR and Cx45 gap junction channels expressed in oocytes.
Functional interaction between CFTR and the sodium-phosphate co-transport type 2a in Xenopus laevis oocytes.
Functional interaction between TRP4 and CFTR in mouse aorta endothelial cells.
Functional interaction of CFTR and ENaC in sweat glands.
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.
Functional interactions of HCO3- with cystic fibrosis transmembrane conductance regulator.
Functional ion channels in pulmonary alveolar type I cells support a role for type I cells in lung ion transport.
Functional modifications of Acid-sensing ion channels by ligand-gated chloride channels.
Functional organization of cytoplasmic portals controlling access to the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel pore.
Functional reconstitution and channel activity measurements of purified wildtype and mutant CFTR protein.
Functional rescue of an ABCB11 mutant by ivacaftor: A new targeted pharmacotherapy approach in bile salt export pump deficiency.
Functional roles of nonconserved structural segments in CFTR's NH2-terminal nucleotide binding domain.
Functional roles of the nucleotide-binding folds in the activation of the cystic fibrosis transmembrane conductance regulator.
Functional stability of CFTR depends on tight binding of ATP at its degenerate ATP-binding site.
Functional Stability of Rescued {Delta}F508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Airway Epithelial Cells.
Functional Vacuolar ATPase (V-ATPase) proton pumps traffic to the enterocyte brush border membrane and require CFTR.
Functions of the cystic fibrosis transmembrane conductance regulator protein.
G protein G alpha i-2 inhibits outwardly rectifying chloride channels in human airway epithelial cells.
G-protein regulation of outwardly rectified epithelial chloride channels incorporated into planar bilayer membranes.
G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects.
G551D Mutation Impairs Protein Kinase A (PKA)-dependent Activation of CFTR Channel that can be Restored by Novel Gain-Of-Function (GOF) Mutations.
G970R-CFTR Mutation (c.2908G>C) Results Predominantly in a Splicing Defect.
GABA induces thee differentiation of small into large cholangiocytes by activation of Ca(2+) /CaMK I-dependent adenylyl cyclase 8.
Gastric secretion.
Gastro-intestinal manifestations in cystic fibrosis patients.
Gastroduodenal mucosal alkaline secretion and mucosal protection.
Gastrointestinal complications of cystic fibrosis.
Gastrointestinal manifestations in cystic fibrosis.
Gastrointestinal Pathology in Juvenile and Adult CFTR-Knockout Ferrets.
Gastrointestinal pathophysiology and nutrition in cystic fibrosis.
Gating of amiloride-sensitive Na(+) channels: subunit-subunit interactions and inhibition by the cystic fibrosis transmembrane conductance regulator.
Gating of cystic fibrosis transmembrane conductance regulator chloride channels by adenosine triphosphate hydrolysis. Quantitative analysis of a cyclic gating scheme.
Gating of the CFTR Cl\#8722; channel by ATP-driven nucleotide-binding domain dimerisation.
Gender-sensitive association of CFTR gene mutations and 5T allele emerging from a large survey on infertility.
Gender-specific protection of estrogen against gastric acid-induced duodenal injury: stimulation of duodenal mucosal bicarbonate secretion.
Gene and cell therapy for cystic fibrosis: from bench to bedside.
Gene delivery to the lungs: pulmonary gene therapy for cystic fibrosis.
Gene expression after freshwater transfer in gills and opercular epithelia of killifish: insight into divergent mechanisms of ion transport.
Gene modifiers of lung disease.
Gene SNPs and mutations in clinical genetic testing: haplotype-based testing and analysis.
Gene targeting of a CFTR allele in HT29 human epithelial cells.
Gene therapy for cystic fibrosis airway disease- is clinical success imminent?
Gene therapy for cystic fibrosis by means of aerosol.
Gene therapy for cystic fibrosis lung disease: current status and future perspectives.
Gene Therapy for Cystic Fibrosis Paved the Way for the Use of Adeno-Associated Virus in Gene Therapy.
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.
Gene therapy for cystic fibrosis.
Gene therapy for cystic fibrosis: a potential cure.
Gene Therapy for Cystic Fibrosis: Progress and Challenges of Genome Editing.
Gene therapy for cystic fibrosis: which postman, which box?
Gene therapy for the respiratory manifestations of cystic fibrosis.
Gene therapy for the treatment of cystic fibrosis.
Gene therapy in a xenograft model of cystic fibrosis lung corrects chloride transport more effectively than the sodium defect.
Gene therapy in 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.
Gene transfer into cystic fibrosis airway epithelial cells.
Gene transfer into the fetal primate: evidence for the secretion of transgene product.
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.
Gene transfer to human fetal pulmonary tissue developed in immunodeficient SCID mice.
Gene transfer to the tracheobronchial tree: implications for fetal gene therapy for cystic fibrosis.
Gene-specific MicroRNA antagonism protects against HIV Tat and TGF-?-mediated suppression of CFTR mRNA and function.
General anesthetic octanol and related compounds activate wild-type and delF508 cystic fibrosis chloride channels.
Generation and phenotype of cell lines derived from CF and non-CF mice that carry the H-2K(b)-tsA58 transgene.
Generation of a conditional null allele for Cftr in mice.
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.
Generation of an induced pluripotent stem cell line (MHHi018-A) from a patient with Cystic Fibrosis carrying p.Asn1303Lys (N1303K) mutation.
Generation of cAMP-activated chloride currents by expression of CFTR.
Generation of functional cholangiocyte-like cells from human pluripotent stem cells and HepaRG cells.
Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study.
Generation of Induced Progenitor-like Cells from Mature Epithelial Cells Using Interrupted Reprogramming.
Generation of KCL021 research grade human embryonic stem cell line carrying a ?F508 mutation in the CFTR gene.
Generation of KCL029 research grade human embryonic stem cell line carrying a mutation in WAS gene.
Generation of multiciliated cells in functional airway epithelia from human induced pluripotent stem cells.
Generation of Novel AAV Variants by Directed Evolution for Improved CFTR Delivery to Human Ciliated Airway Epithelium.
Genes in the vicinity of CFTR modulate the cystic fibrosis phenotype in highly concordant or discordant F508del homozygous sib pairs.
Genetic analysis of rwandan patients with cystic fibrosis-like symptoms: identification of novel cystic fibrosis transmembrane conductance regulator and epithelial sodium channel gene variants.
Genetic analysis of the bicarbonate secreting anion exchanger SLC26A6 in chronic pancreatitis.
Genetic and biochemical markers of obstructive lung disease in the general population.
Genetic and clinical features of false-negative infants in a neonatal screening programme for cystic fibrosis.
Genetic and phenotypic heterogeneity in tropical calcific pancreatitis.
Genetic and phenotypic traits of children and adolescents with cystic fibrosis in Southern Brazil.
Genetic and physical mapping of the ovine cystic fibrosis gene.
Genetic aspects of chronic pancreatitis.
Genetic aspects of pancreatitis.
Genetic basis of variable exon 9 skipping in cystic fibrosis transmembrane conductance regulator mRNA.
Genetic characterisation of circulating fetal cells allows non-invasive prenatal diagnosis of cystic fibrosis.
Genetic comparisons of patients with cystic fibrosis with or without meconium ileus. Clinical Centers of the French CF Registry.
Genetic concerns for the subfertile male in the era of ICSI.
Genetic cystic fibrosis transmembrane regulator 4016insT D1152H compound heterozygosity and male infertility: an Italian case report.
Genetic defects in human azoospermia.
Genetic deletion of keratin 8 corrects the altered bone formation and osteopenia in a mouse model of cystic fibrosis.
Genetic determinants and epidemiology of cystic fibrosis-related diabetes: results from a British cohort of children and adults.
Genetic diagnosis in infertile men with numerical and constitutional sperm abnormalities.
Genetic diagnostics of male infertility in clinical practice.
Genetic diseases of the seminal ducts.
Genetic Evaluation of Children with Idiopathic Recurrent Acute Pancreatitis.
Genetic evaluation of male infertility.
Genetic factors in chronic pancreatitis; implications for diagnosis, management and prognosis.
Genetic factors in pancreatitis.
Genetic investigations of CFTR mutations in congenital absence of vas deferens, uterus, and vagina as a cause of infertility.
Genetic issues in pediatric pancreatitis.
Genetic markers of male infertility: Y chromosome microdeletions and cystic fibrosis transmembrane conductance gene mutations.
Genetic modification of cystic fibrosis with ?F508 mutation of CFTR gene using the CRISPR system in peripheral blood mononuclear cells.
Genetic modifiers of lung disease in cystic fibrosis.
Genetic polymorphisms in alcoholic pancreatitis.
Genetic Polymorphisms in the Host and COVID-19 Infection.
Genetic risk factors for chronic obstructive pulmonary disease.
Genetic risk factors for pancreatic disorders.
Genetic risk factors in infertile men with severe oligozoospermia and azoospermia.
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.
Genetic screening in Italian infertile couples undergoing intrauterine insemination and in vitro fertilization techniques: a multicentric study.
Genetic testing in acute and chronic pancreatitis.
Genetic variation within the ovine cystic fibrosis transmembrane conductance regulator gene.
Genetic, andrological and clinical characteristics of patients with congenital bilateral absence of the vas deferens.
Genetic, cell biological, and clinical interrogation of the CFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention.
Genetic, epidemiological, and clinical aspects of hereditary pancreatitis: a population-based cohort study in Denmark.
Genetics and pancreatic disease.
Genetics of CFTR and male infertility.
Genetics of chronic obstructive pulmonary disease, beyond a1-antitrypsin deficiency.
Genetics of chronic pancreatitis.
Genetics of chronic rhinosinusitis: State of the field and directions forward.
Genetics of cystic fibrosis.
Genetics of cystic fibrosis: CFTR mutation classifications toward genotype-based CF therapies.
Genetics of Cystic Fibrosis: Clinical Implications.
Genistein activates CFTR Cl- channels via a tyrosine kinase- and protein phosphatase-independent mechanism.
Genistein activates CFTR-mediated Cl(-) secretion in the murine trachea and colon.
Genistein and tyrphostin 47 stimulate CFTR-mediated Cl- secretion in T84 cell monolayers.
Genistein directly induces cardiac CFTR chloride current by a tyrosine kinase-independent and protein kinase A-independent pathway in guinea pig ventricular myocytes.
Genistein improves regulatory interactions between G551D-cystic fibrosis transmembrane conductance regulator and the epithelial sodium channel in Xenopus oocytes.
Genistein modifies the activation kinetics and magnitude of phosphorylated wild-type and G551D-CFTR chloride currents.
Genistein restores functional interactions between Delta F508-CFTR and ENaC in Xenopus oocytes.
Genistein stimulates duodenal HCO(3)(-) secretion through PI3K pathway in mice.
Genistein-Calcitriol Mitigates Hyperosmotic Stress-Induced TonEBP, CFTR Dysfunction, VDR Degradation and Inflammation in Dry Eye Disease.
Genistein-induced fluid accumulation in ovariectomised rats' uteri is associated with increased cystic fibrosis transmembrane regulator expression.
Genome-wide analysis of MicroRNA-messenger RNA interactome in ex-vivo gill filaments, Anguilla japonica.
Genome-wide association study for poor sperm motility in Holstein-Friesian bulls.
Genomewide association analysis of respiratory syncytial virus infection in mice.
Genomic DNA sequence of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Genomic rearrangements in the CFTR gene: extensive allelic heterogeneity and diverse mutational mechanisms.
Genomic sequence analysis of Fugu rubripes CFTR and flanking genes in a 60 kb region conserving synteny with 800 kb of human chromosome 7.
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?
Genotype patterns for mutations of the cystic fibrosis transmembrane conductance regulator gene: a retrospective descriptive study from Saudi Arabia.
Genotype-phenotype correlation and functional studies in patients with cystic fibrosis bearing CFTR complex alleles.
Genotype-phenotype correlation in 9 patients with tropical pancreatitis and identified gene mutations.
Genotype-phenotype correlation in cystic fibrosis patients.
Genotype-phenotype correlations in cystic fibrosis: clinical severity of mutation S549R(T-->G).
Genotype-phenotype relationship for five CFTR mutations frequently identified in western France.
Genotype-phenotype relationships in a cohort of adult cystic fibrosis patients.
Genotype/phenotype correlation of the G85E mutation in a large cohort of cystic fibrosis patients.
Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis.
Genotyping microarray for the detection of more than 200 CFTR mutations in ethnically diverse populations.
Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations.
Geographic distribution of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Saudi Arabia.
Geographic distribution of cystic fibrosis transmembrane regulator gene mutations in Saudi Arabia.
Geographic heterogeneity of 4 common worldwide cystic fibrosis non-DF508 mutations in Brazil.
Germline mutations and gene polymorphism associated with human pancreatitis.
Germline mutations in CFTR and PSTI genes in chronic pancreatitis patients.
Gestational and tissue-specific regulation of C1C-2 chloride channel expression.
GFP-tagged CFTR transgene is functional in the G551D cystic fibrosis mouse colon.
Glandular Proteome Identifies Antiprotease Cystatin C as a Critical Modulator of Airway Hydration and Clearance.
Glial cells express multiple ATP binding cassette proteins which are involved in ATP release.
Glibenclamide blockade of CFTR chloride channels.
Glibenclamide induces apoptosis through inhibition of cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channels and intracellular Ca(2+) release in HepG2 human hepatoblastoma cells.
Glibenclamide inhibits BK polyomavirus infection in kidney cells through CFTR blockade.
Glibenclamide stimulates fluid secretion in rodent cholangiocytes through a cystic fibrosis transmembrane conductance regulator-independent mechanism.
Glibenclamide, an ATP-sensitive K+ channel blocker, inhibits cardiac cAMP-activated Cl- conductance.
Global assessment of the integrated stress response in CF patient-derived airway and intestinal tissues.
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.
GLPG1837, a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1).
Glucocorticoid dexamethasone regulates the differentiation of mouse conducting airway epithelial progenitor cells.
Glucocorticoids and myosin5b loss of function induce heightened PKA signaling in addition to membrane traffic defects.
Glucocorticoids Distinctively Modulate the CFTR Channel with Possible Implications in Lung Development and Transition into Extrauterine Life.
Glucose homeostasis and genotype-phenotype interplay in cystic fibrosis patients with CFTR gene deltaF508 mutation.
Glucose sensing mechanisms in hypothalamic cell models: glucose inhibition of AgRP synthesis and secretion.
Glutamate acts at NMDA receptors on fresh bovine and on cultured human retinal pigment epithelial cells to trigger release of ATP.
Glutathione levels and BAX activation during apoptosis due to oxidative stress in cells expressing wild-type and mutant cystic fibrosis transmembrane conductance regulator.
Glutathione permeability of CFTR.
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.
Glycosylation and the cystic fibrosis transmembrane conductance regulator.
Glycosylation status of endogenous CFTR does not affect cAMP-stimulated Cl- secretion in epithelial cells.
GM1 as Adjuvant of Innovative Therapies for Cystic Fibrosis Disease.
Gout-causing Q141K mutation in ABCG2 leads to instability of the nucleotide-binding domain and can be corrected with small molecules.
Gp78 Cooperates with RMA1 in Endoplasmic Reticulum-associated Degradation of CFTR{Delta}F508.
Gramicidin-perforated patch analysis on HCO3- secretion through a forskolin-activated anion channel in rat parotid intralobular duct cells.
Green fluorescent protein-based halide indicators with improved chloride and iodide affinities.
Growth and nutritional status in children and adolescents with cystic fibrosis.
Growth deficits in cystic fibrosis mice begin in utero prior to IGF-1 reduction.
Growth of putative progenitors of type II pneumocytes in culture of human cystic fibrosis alveoli.
Guanylin activates Cl- secretion into the lumen of seawater eel intestine via apical Cl- channel under simulated in vivo conditions.
Guanylin and functional coupling proteins in the human salivary glands and gland tumors : expression, cellular localization, and target membrane domains.
Guanylin and related peptides.
Guanylin and uroguanylin in the parotid and submandibular glands: potential intrinsic regulators of electrolyte secretion in salivary glands.
Guanylin in the human pancreas: a novel luminocrine regulatory pathway of electrolyte secretion via cGMP and CFTR in the ductal system.
Guanylin mRNA expression in human intestine and colorectal adenocarcinoma.
Guanylin peptides regulate electrolyte and fluid transport in the Gulf toadfish (Opsanus beta) posterior intestine.
Guanylin regulates chloride secretion in the human gallbladder via the bile fluid.
Guanylin strongly stimulates rat duodenal HCO3- secretion: proposed mechanism and comparison with other secretagogues.
Guidelines for the clinical management and follow-up of infants with inconclusive cystic fibrosis diagnosis through newborn screening.
Gut-Ex-Vivo system as a model to study gluten response in celiac disease.
H2O2 Stimulates Cystic Fibrosis Transmembrane Conductance Regulator through an Autocrine Prostaglandin Pathway, Using Multidrug-Resistant Protein-4.
H5N1 Virus Haemagglutinin Inhibition of cAMP-Dependent CFTR via TLR4-Mediated Janus Tyrosine Kinase 3 Activation Exacerbates Lung Inflammation.
Halide permeation in wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride channels.
HALT-D: A Phase II Evaluation of Crofelemer for the Prevention and Prophylaxis of Diarrhea in Patients With Breast Cancer on Pertuzumab-Based Regimens.
Haplotype analysis of the CFTR gene on normal and mutant CFTR genes.
Harnessing Neutrophil Survival Mechanisms during Chronic Infection by Pseudomonas aeruginosa: Novel Therapeutic Targets to Dampen Inflammation in Cystic Fibrosis.
HCO(3)(-)-dependent soluble adenylyl cyclase activates cystic fibrosis transmembrane conductance regulator in corneal endothelium.
HCO3-dependent pHi regulation in tracheal epithelial cells.
Head growth in cystic fibrosis following early diagnosis by neonatal screening.
Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?
Heat treatment of thioredoxin fusions increases the purity of ?-helical transmembrane protein constructs.
Heat-stable enterotoxin of Escherichia coli (STa) can stimulate duodenal HCO3(-) secretion via a novel GC-C- and CFTR-independent pathway.
HEK-293 cells expressing the cystic fibrosis transmembrane conductance regulator (CFTR): a model for studying regulation of Cl- transport.
Helicobacter pylori infection downregulates duodenal CFTR and SLC26A6 expressions through TGF? signaling pathway.
Hematopoietic Stem/Progenitor Cells Express Functional Mitochondrial Energy-Dependent Cystic Fibrosis Transmembrane Conductance Regulator.
Hepatic manifestations of cystic fibrosis.
Hepatobiliary complications of cystic fibrosis.
Hepatobiliary disease in patients with cystic fibrosis.
Herbal components of Japanese Kampo medicines exert laxative actions in colonic epithelium cells via activation of BK and CFTR channels.
Hereditary chronic pancreatitis.
Hereditary pancreatitis for the endoscopist.
Hereditary pancreatitis in childhood: course of disease and complications.
Hereditary pancreatitis in Japan: a review of pancreatitis-associated gene mutations.
Heritability of Respiratory Infection with Pseudomonas aeruginosa in Cystic Fibrosis.
Hesperidin stimulates cystic fibrosis transmembrane conductance regulator-mediated chloride secretion and ciliary beat frequency in sinonasal epithelium.
Heterogeneity in mouse spasmolytic polypeptide-expressing metaplasia lineages identifies markers of metaplastic progression.
Heterogeneity in the severity of cystic fibrosis and the role of CFTR gene mutations.
Heterogeneity of phenotype in two cystic fibrosis patients homozygous for the CFTR exon 11 mutation G551D.
Heterogeneity of reproductive tract abnormalities in men with absence of the vas deferens: role of cystic fibrosis transmembrane conductance regulator gene mutations.
Heterogeneity of the cystic fibrosis phenotype in a large kindred family in Qatar with cystic fibrosis mutation (I1234V).
Heterogeneous Spectrum of CFTR Gene Mutations in Korean Patients with 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.
Heterogenous spectrum of CFTR gene mutations in Indian patients with congenital absence of vas deferens.
Heterologous expression of delta F508 CFTR results in decreased sialylation of membrane glycoconjugates.
Heterologous expression systems for study of cystic fibrosis transmembrane conductance regulator.
Heterologous regulation of anion transporters by menthol in human airway epithelial cells.
Heterotrimeric G proteins, vesicle trafficking, and CFTR Cl- channels.
Heterozygosity for the F508del mutation in the cystic fibrosis transmembrane conductance regulator anion channel attenuates influenza severity.
Heterozygous Cystic Fibrosis Transmembrane Regulator Gene Missense Variants Are Associated With Worse Cardiac Function in Patients With Duchenne Muscular Dystrophy.
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.
High affinity ATP/ADP analogues as new tools for studying CFTR gating.
High allelic heterogeneity between Afro-Brazilians and Euro-Brazilians impacts cystic fibrosis genetic testing.
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.
High density lipoprotein promoting proliferation and migration of type II alveolar epithelial cells during inflammation state.
High frequency of (TG)mTn variant tracts in the cystic fibrosis transmembrane conductance regulator gene in men with high semen viscosity.
High frequency of cystic fibrosis transmembrane regulator mutation L997F in patients with recurrent idiopathic pancreatitis and in newborns with hypertrypsinemia.
High fructose causes cardiac hypertrophy via mitochondrial signaling pathway.
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).
High level of CFTR expression is associated with tumor aggression and knockdown of CFTR suppresses proliferation of ovarian cancer in vitro and in vivo.
High-affinity activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening.
High-density multiplex detection of nucleic acid sequences: oligonucleotide ligation assay and sequence-coded separation.
High-efficiency transfer of cystic fibrosis transmembrane conductance regulator cDNA into cystic fibrosis airway cells in culture using lactosylated polylysine as a vector.
High-Efficiency, Selection-free Gene Repair in Airway Stem Cells from Cystic Fibrosis Patients Rescues CFTR Function in Differentiated Epithelia.
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.
High-fat diet modifies the PPAR-? pathway leading to disruption of microbial and physiological ecosystem in murine small intestine.
High-Potency Phenylquinoxalinone Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activators.
High-resolution imaging of the actin cytoskeleton and epithelial sodium channel, CFTR, and aquaporin-9 localization in the vas deferens.
High-Throughput Screening for Modulators of CFTR Activity Based on Genetically Engineered Cystic Fibrosis Disease-Specific iPSCs.
High-Throughput Screening for Readthrough Modulators of CFTR PTC Mutations.
High-throughput screening identifies FAU protein as a regulator of mutant cystic fibrosis transmembrane conductance regulator channel.
Higher proportion of intact exon 9 CFTR mRNA in nasal epithelium compared with vas deferens.
Highlights of the 28(th) North American Cystic Fibrosis Conference 2014.
Histamine 1 receptor-G??-cAMP/PKA-CFTR pathway mediates the histamine-induced resetting of the suprachiasmatic circadian clock.
Histological and genetic analysis and risk assessment for chromosomal aberration after ICSI for patients presenting with CBAVD.
History of cryptorchidism and ejaculate volume as simple predictors for the presence of testicular sperm.
HIV Infects Bronchial Epithelium and Suppresses Components of the Mucociliary Clearance Apparatus.
HLA class II polymorphism in cystic fibrosis. A possible modifier of pulmonary phenotype.
Homogeneity of Aerosol Deposition and Mucociliary Clearance are Improved Following Ivacaftor Treatment in Cystic Fibrosis.
Homology Requirements for Efficient, Footprintless Gene Editing at the CFTR Locus in Human iPSCs with Helper-dependent Adenoviral Vectors.
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.
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.
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.
Homozygous Deletion of the CFTR Gene Caused by Interstitial Maternal Isodisomy in a Peruvian Child with Cystic Fibrosis.
Hook2 contributes to aggresome formation.
Hormonal control of the renal immune response and antibacterial host defense by arginine vasopressin.
Hormonal regulation of cardiac cystic fibrosis transmembrane conductance regulator chloride channels.
Host mucin glycosylation plays a role in bacterial adhesion in lungs of individuals with cystic fibrosis.
Host resistance to lung infection mediated by major vault protein in epithelial cells.
House dust mite extract activates apical Cl(-) channels through protease-activated receptor 2 in human airway epithelia.
How Clinically Efficient Is Lumacaftor/Ivacaftor for Cystic Fibrosis Patients? An Updated Literature Review.
How do cystic fibrosis transmembrane conductance regulator mutations produce lung disease?
How mutant CFTR may contribute to Pseudomonas aeruginosa infection in cystic fibrosis.
How Phosphorylation and ATPase Activity Regulate Anion Flux though the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
How phosphorylation and ATPase activity regulate anion flux through the cystic fibrosis transmembrane conductance regulator (CFTR).
How the airway smooth muscle in cystic fibrosis reacts in proinflammatory conditions: implications for airway hyper-responsiveness and asthma in cystic fibrosis.
Hsp104 facilitates the endoplasmic-reticulum-associated degradation of disease-associated and aggregation-prone substrates.
Hsp70 molecular chaperone facilitates endoplasmic reticulum-associated protein degradation of cystic fibrosis transmembrane conductance regulator in yeast.
Hsp90 and client protein maturation.
Human alveolar type II cells secrete and absorb liquid in response to local nucleotide signaling.
Human amnion epithelial cells induced to express functional cystic fibrosis transmembrane conductance regulator.
Human CFTR gene sequences in regions flanking exon 10: a simple repeat sequence polymorphism in intron 9.
Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance.
Human cystic fibrosis monocyte derived macrophages display no defect in acidification of phagolysosomes when measured by optical nanosensors.
Human cystic fibrosis transmembrane conductance regulator directed to respiratory epithelial cells of transgenic mice.
Human Enteroids as a Model of Upper Small Intestinal Ion Transport Physiology and Pathophysiology.
Human epithelial cystic fibrosis transmembrane conductance regulator without exon 5 maintains partial chloride channel function in intracellular membranes.
Human extrahepatic and intrahepatic cholangiocyte organoids show region-specific differentiation potential and model cystic fibrosis-related bile duct disease.
Human fetal trachea-SCID mouse xenografts: efficacy of vesicular stomatitis virus-G pseudotyped lentiviral-mediated gene transfer.
Human Genetic Variation Influences Enteric Fever Progression.
Human Genetics Society of Australasia position statement: population-based carrier screening for 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.
Human lymphocytes transcribe the cystic fibrosis transmembrane conductance regulator gene and exhibit CF-defective cAMP-regulated chloride current.
Human Nasal Epithelial Organoids for Therapeutic Development in Cystic Fibrosis.
Human pluripotent stem cell-derived acinar/ductal organoids generate human pancreas upon orthotopic transplantation and allow disease modelling.
Human Sperm Capacitation Involves the Regulation of the Tyr-Phosphorylation Level of the Anion Exchanger 1 (AE1).
Human, Drosophila, and C.elegans TDP43: nucleic acid binding properties and splicing regulatory function.
Human-mouse cystic fibrosis transmembrane conductance regulator (CFTR) chimeras identify regions that partially rescue CFTR-?F508 processing and alter its gating defect.
Human-specific cystic fibrosis transmembrane conductance regulator antibodies detect in vivo gene transfer to ovine airways.
Humoral and cellular immune responses of nonhuman primates to long-term repeated lung exposure to Ad2/CFTR-2.
Huqi San-Evoked Rat Colonic Anion Secretion through Increasing CFTR Expression.
Hybrid Nonviral/Viral Vector Systems for Improved piggyBac DNA Transposon In Vivo Delivery.
Hybridization of glass-tethered oligonucleotide probes to target strands preannealed with labeled auxiliary oligonucleotides.
Hydrator Therapies for Chronic Bronchitis. Lessons from Cystic Fibrosis.
Hydrogen peroxide stimulates chloride secretion in primary inner medullary collecting duct cells via mPGES-1-derived PGE2.
Hydrogen Sulfide Facilitates Vaginal Lubrication by Activation of Epithelial ATP-Sensitive K(+) Channels and Cystic Fibrosis Transmembrane Conductance Regulator.
Hydrogen sulfide stimulates CFTR in Xenopus oocytes by activation of the cAMP/PKA signalling axis.
Hydrogen sulfide: A novel signaling molecule in the central nervous system.
Hydrolytic and nonhydrolytic interactions in the ATP regulation of CFTR Cl- conductance.
Hyper-osmolarity and calcium chelation: Effects on cystic fibrosis mucus.
Hyperabsorption of Na+ and raised Ca(2+)-mediated Cl- secretion in nasal epithelia of CF mice.
Hyperacidification of cellubrevin endocytic compartments and defective endosomal recycling in cystic fibrosis respiratory epithelial cells.
Hypercapnia modulates cAMP signalling and cystic fibrosis transmembrane conductance regulator-dependent anion and fluid secretion in airway epithelia.
Hyperechogenic fetal bowel: counseling difficulties.
Hyperexpression of recombinant CFTR in heterologous cells alters its physiological properties.
Hypertonic saline increases lung epithelial lining fluid glutathione and thiocyanate: two protective CFTR-dependent thiols against oxidative injury.
Hypertonic saline releases the attached small intestinal cystic fibrosis mucus.
Hypertonic saline solutions do not influence the solubility of sputum from secretor and non-secretor cystic fibrosis patients.
Hypertonic saline treatment of cystic fibrosis.
Hypochloremia and hyponatremia as the initial presentation of cystic fibrosis in three adults.
Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands.
Hypothesis: vitamin E complements polyunsaturated fatty acids in essential fatty acid deficiency in cystic fibrosis.
Hypothetical framework for enhanced renal tubular secretion of drugs in cystic fibrosis.
Hypoxia increases corneal cell expression of CFTR leading to increased Pseudomonas aeruginosa binding, internalization, and initiation of inflammation.
Hypoxia inducible factor-1 (HIF-1)-mediated repression of cystic fibrosis transmembrane conductance regulator (CFTR) in the intestinal epithelium.
Hypoxia Inhibits Colonic Ion Transport via Activation of AMP Kinase.
Ibuprofen rescues mutant cystic fibrosis transmembrane conductance regulator trafficking.
ICAM-1 expression on conjunctival epithelial cells in patients with cystic fibrosis.
Identical intragenic microsatellite haplotype found in cystic fibrosis chromosomes bearing mutation G551D in Irish, English, Scottish, Breton and Czech patients.
Identification and characterization of CFTR gene mutations in Indian CF patients.
Identification and Characterization of Novel CFTR Potentiators.
Identification and developmental expression of the Xenopus laevis cystic fibrosis transmembrane conductance regulator gene.
Identification and localization of a dogfish homolog of human cystic fibrosis transmembrane conductance regulator.
Identification and membrane localization of electrogenic sodium bicarbonate cotransporters in Calu-3 cells.
Identification and partial characterization of a domain in CFTR that may bind cyclic nucleotides directly.
Identification and regulation of the cystic fibrosis transmembrane conductance regulator-generated chloride channel.
Identification of a cystic fibrosis mutation W19X in Tunisia.
Identification of a new cystic fibrosis transmembrane regulator mutation in a severely affected patient.
Identification of a Novel 5' Alternative CFTR mRNA Isoform in a Patient with Nasal Polyposis and CFTR Mutations.
Identification of a Novel Frame-Shift Mutation in PRSS1 Gene in Han Patients with Autoimmune Pancreatitis.
Identification of a novel functional miR-143-5p recognition element in the Cystic Fibrosis Transmembrane Conductance Regulator 3'UTR.
Identification of a novel large deletion and other copy number variations in the CFTR gene in patients with Cystic Fibrosis from a multiethnic population.
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.
Identification of a novel mutation of CFTR gene in a Korean patient with 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.
Identification of a novel post-hydrolytic state in CFTR gating.
Identification of a novel SERPINA-1 mutation causing alpha-1 antitrypsin deficiency in a patient with severe bronchiectasis and pulmonary embolism.
Identification of a novel water soluble activator of wild-type and F508del CFTR: GPact-11a.
Identification of a protein that confers calcitonin gene-related peptide responsiveness to oocytes by using a cystic fibrosis transmembrane conductance regulator assay.
Identification of a region of strong discrimination in the pore of CFTR.
Identification of a second blocker binding site at the cytoplasmic mouth of the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Identification of an 11T allele in the polypyrimidine tract of intron 8 of the CFTR gene.
Identification of an Anti-Inflammation Protein, Annexin A1, in Tendon Derived Stem Cells (TDSCs) of Cystic Fibrosis Mice: A Comparative Proteomic Analysis.
Identification of an ion channel-forming motif in the primary structure of CFTR, the cystic fibrosis chloride channel.
Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator.
Identification of CFTR activators and inhibitors: chance or design?
Identification of CFTR, PRSS1, and SPINK1 mutations in 381 patients with pancreatitis.
Identification of Compounds That Promote Readthrough of Premature Termination Codons in the CFTR.
Identification of cystic fibrosis transmembrane conductance regulator channel-lining residues in and flanking the M6 membrane-spanning segment.
Identification of cystic fibrosis transmembrane conductance regulator in renal endosomes.
Identification of eight mutations and three sequence variations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Identification of four new mutations in the cystic fibrosis transmembrane conductance regulator gene: I148T, L1077P, Y1092X, 2183AA-->G.
Identification of four novel mutations in the cystic fibrosis transmembrane conductance regulator gene: E664X, 2113delA, 306delTAGA, and delta M1140.
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.
Identification of genes and pathways in esophageal adenocarcinoma using bioinformatics analysis.
Identification of GLPG/ABBV-2737, a Novel Class of Corrector, Which Exerts Functional Synergy With Other CFTR Modulators.
Identification of key pathways and genes in Barrett's esophagus using integrated bioinformatics methods.
Identification of molecular determinants that modulate trafficking of DeltaF508 CFTR, the mutant ABC transporter associated with cystic fibrosis.
Identification of mutations in exons 1 through 8 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Identification of natural coumarin compounds that rescue defective DeltaF508-CFTR chloride channel gating.
Identification of new small molecule inhibitors of cystic fibrosis transmembrane conductance regulator protein: in vitro and in vivo studies.
Identification of novel and rare mutations in California Hispanic and African American cystic fibrosis patients.
Identification of novel mutations in Arabs with cystic fibrosis and their impact on the cystic fibrosis transmembrane regulator mutation detection rate in Arab populations.
Identification of patients with genetic risk factors of pancreatitis: impact on treatment and cancer prevention.
Identification of positive charges situated at the outer mouth of the CFTR chloride channel pore.
Identification of Potential Leukocyte Biomarkers Related to Drug Recovery of CFTR: Clinical Applications in 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.
Identification of resveratrol oligomers as inhibitors of cystic fibrosis transmembrane conductance regulator by high-throughput screening of natural products from chinese medicinal plants.
Identification of revertants for the cystic fibrosis delta F508 mutation using STE6-CFTR chimeras in yeast.
Identification of RL-TGR, a coreceptor involved in aversive chemical signaling.
Identification of six mutations (R31L, 441delA, 681delC, 1461ins4, W1089R, E1104X) in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Identification of specific sites in human P-glycoprotein phosphorylated by protein kinase C.
Identification of synergistic combinations of F508del cystic fibrosis transmembrane conductance regulator (CFTR) modulators.
Identification of the 5T-12TG allele of the cystic fibrosis transmembrane conductance regulator gene in hypertrypsinaemic newborns.
Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequences.
Identification of the cofilin-binding sites in the large cytoplasmic domain of Na,K-ATPase.
Identification of the commonest cystic fibrosis transmembrane regulator gene DeltaF508 mutation: evaluation of PCR--single-strand conformational polymorphism and polyacrylamide gel electrophoresis.
Identification of the cystic fibrosis transmembrane conductance regulator domains that are important for interactions with ROMK2.
Identification of the five most common cystic fibrosis mutations in single cells using a rapid and specific differential amplification system.
Identification of the human cortactin-binding protein-2 gene from the autism candidate region at 7q31.
Identification of the linkage of mutations causing cystic fibrosis to different alleles of a tetranucleotide repeat in intron 6a of the CFTR gene.
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.
Identification of the major phosphorylation domain of murine mdr1b P-glycoprotein. Analysis of the protein kinase A and protein kinase C phosphorylation sites.
Identification of the Novel TMEM16A Inhibitor Dehydroandrographolide and Its Anticancer Activity on SW620 Cells.
Identification of three novel mutations (457 TAT-->G, D192G, Q685X) in the Slovenian CF patients.
Identification of three novel mutations in the CFTR gene using temperature-optimized non-radioactive conditions for SSCP analysis.
Identification of three novel mutations in the cystic fibrosis transmembrane conductance regulator gene in Argentinian CF patients.
Identification of transport abnormalities in duodenal mucosa and duodenal enterocytes from patients with cystic fibrosis.
Idiopathic CD4+ T lymphocytopenia presenting as mycetoma in a patient with a mutation in the cystic fibrosis transmembrane regulator gene.
IFN{gamma} Regulates Retinal Pigment Epithelial Fluid Transport.
IGF binding protein-3 treatment alters intestinal cell proliferation but not body weight of adult cystic fibrosis transmembrane conductance regulator deficient mice.
IL-17 primes airway epithelial cells lacking functional Cystic Fibrosis Transmembrane conductance Regulator (CFTR) to increase NOD1 responses.
IL-4 is a potent modulator of ion transport in the human bronchial epithelium in vitro.
IL-8 inhibits cAMP-stimulated alveolar epithelial fluid transport via a GRK2/PI3K-dependent mechanism.
Image-based ?-adrenergic sweat rate assay captures minimal cystic fibrosis transmembrane conductance regulator function.
Imaging CFTR in its native environment.
Imaging CFTR: a tail to tail dimer with a central pore.
Immortalization of bovine pancreatic duct epithelial cells.
Immortalization of subpopulations of respiratory epithelial cells from transgenic mice bearing SV40 large T antigen.
Immortalized bovine pancreatic duct cells become tumorigenic after transfection with mutant k-ras.
Immortalized epithelial cells from human autosomal dominant polycystic kidney cysts.
Immortalized intrahepatic mouse biliary epithelial cells: immunologic characterization and immunogenicity.
Immune mediators regulate CFTR expression through a bifunctional airway-selective enhancer.
Immune regulation of cystic fibrosis transmembrane regulator.
Immune response of polarized cystic fibrosis airway epithelial cells infected with Influenza A virus.
Immune responses in Cystic Fibrosis; are they intrinsically defective?
Immuno and functional characterization of CFTR in submandibular and pancreatic acinar and duct cells.
Immunocytochemical analysis reveals differences between the subcellular localization of normal and delta Phe508 recombinant cystic fibrosis transmembrane conductance regulator.
Immunohistochemical analysis of the distribution of molecules involved in ionic and pH regulation in the lancelet Branchiostoma floridae (Hubbs, 1922).
Immunohistochemical and electron microscopic characterization of brush cells of the rat cecum.
Immunohistochemical Detection of Markers for Translational Studies of Lung Disease in Pigs and Humans.
Immunohistochemical localization of cystic fibrosis transmembrane conductance regulator in human fetal airway and digestive mucosa.
Immunohistochemical localization of cystic fibrosis transmembrane regulator and clara cell secretory protein in taste receptor cells of rat circumvallate papillae.
Immunohistochemical localization of guanylin in the rat small intestine and colon.
Immunohystochemical analysis of CFTR in normal and disrupted spermatogenesis.
Immunolocalization and regulation of cystic fibrosis transmembrane conductance regulator in the adult rat epididymis.
Immunolocalization of chloride transporters to gill epithelia of euryhaline teleosts with opposite salinity-induced Na(+)/K (+)-ATPase responses.
Immunolocalization of ion transport proteins in human autosomal dominant polycystic kidney epithelial cells.
Immunolocalization of ion-transport proteins to branchial epithelium mitochondria-rich cells in the mudskipper (Periophthalmodon schlosseri).
Immunologic responses to gene transfer into mice via the polymeric immunoglobulin receptor.
Immunomodulation in Cystic Fibrosis: Why and How?
Immunopathology and immunogenetics of allergic bronchopulmonary aspergillosis.
Immunopathology of Airway Surface Liquid Dehydration Disease.
Impact of a cystic fibrosis transmembrane conductance regulator (CFTR) modulator on high-dose ibuprofen therapy in pediatric cystic fibrosis patients.
Impact of Altered Gut Microbiota and Its Metabolites in Cystic Fibrosis.
Impact of CFTR DeltaF508 mutation on prostaglandin E2 production and type IIA phospholipase A2 expression by pulmonary epithelial cells.
Impact of CFTR modulation with Ivacaftor on Gut Microbiota and Intestinal Inflammation.
Impact of CFTR-modulating drugs on GH-IGF-1 axis impairment in adult patients with cystic fibrosis.
Impact of cystic fibrosis on bone health.
Impact of cystic fibrosis transmembrane conductance regulator gene mutation on the occurrence of chronic pancreatitis in Japanese patients.
Impact of Cystic Fibrosis Transmembrane Regulator (CFTR) gene mutations on male infertility.
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.
Impact of high- versus low-risk genotype on sinonasal radiographic disease in cystic fibrosis.
Impact of hypoxia and AMPK on CFTR-mediated bicarbonate secretion in human cholangiocyte organoids.
Impact of mannose binding lectin (MBL) insufficiency on the course of cystic fibrosis: a review and meta-analysis.
Impact of pharmacy services on cystic fibrosis transmembrane conductance regulator modulator prescribing at a pediatric cystic fibrosis center.
Impact of Physical Activity on Cognitive Functions: A New Field for Research and Management of Cystic Fibrosis.
Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth.
Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure.
Impact of the F508del mutation on ovine CFTR, a Cl(-) channel with enhanced conductance and ATP-dependent gating.
Impaired cardiac and peripheral hemodynamic responses to inhaled ??-agonist in cystic fibrosis.
Impaired cell volume regulation in intestinal crypt epithelia of cystic fibrosis mice.
Impaired CFTR-Dependent Amplification of FSH-Stimulated Estrogen Production in Cystic Fibrosis and PCOS.
Impaired pancreatic ductal bicarbonate secretion in 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.
Impaired Renal HCO3- Excretion in Cystic Fibrosis.
Impaired transporter associated with antigen processing (TAP) function attributable to a single amino acid alteration in the peptide TAP subunit TAP1.
Impairment of the ubiquitin-proteasome system by protein aggregation.
Implementation of newborn screening for cystic fibrosis in Norway. Results from the first three years.
Implication of the cystic fibrosis transmembrane conductance regulator gene in infertile family members of Indian CF patients.
Importance of basolateral K+ conductance in maintaining Cl- secretion in murine nasal and colonic epithelia.
Importance of SLC26 Transmembrane Anion Exchangers in Sperm Post-testicular Maturation and Fertilization Potential.
Improved correction of F508del-CFTR biogenesis with a folding facilitator and an inhibitor of protein ubiquitination.
Improved detection of CFTR mutations in Southern California Hispanic CF patients.
Improved detection of cystic fibrosis mutations in infertility patients with DNA sequence analysis.
Improved fluorescence assays to measure the defects associated with F508del-CFTR allow identification of new active compounds.
Improved maturation of CFTR by an ER export signal.
Improved oxygenation promotes CFTR maturation and trafficking in MDCK monolayers.
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.
Improving complex medical care while awaiting next-generation CFTR potentiators and correctors: The current pipeline of therapeutics.
Improving newborn screening for cystic fibrosis using next-generation sequencing technology: a technical feasibility study.
Improving outcomes of infections in cystic fibrosis in the era of CFTR modulator therapy.
In cellulo analyses of the p.Val322Ala mutation on the CFTR protein conformation and activity.
In NF1, CFTR, PER3, CARS and SYT7, alternatively included exons show higher conservation of surrounding intron sequences than constitutive exons.
In silico drug repositioning on F508del-CFTR: A proof-of-concept study on the AIFA library.
In silico prediction and validation of potential therapeutic genes in pancreatic ?-cells associated with type 2 diabetes.
In utero and postnatal VX-770 administration rescues multiorgan disease in a ferret model of cystic fibrosis.
In vitro 3D culture lung model from expanded primary cystic fibrosis human airway cells.
In Vitro Analysis of PDZ-dependent CFTR Macromolecular Signaling Complexes.
In vitro and in vivo functional characterization of gutless recombinant SV40-derived CFTR vectors.
In vitro assessment of variables affecting the efficiency and efficacy of adenovirus-mediated gene transfer to cystic fibrosis airway epithelia.
In vitro evaluation of drug delivery behavior for inhalable amorphous nanoparticle formulations in a human lung epithelial cell model.
In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.
In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study.
In Vitro Recovery of ATP-Sensitive Potassium Channels in {beta}-Cells From Patients With Congenital Hyperinsulinism of Infancy.
In vitro validation of duct differentiation in developing embryonic mouse pancreas.
In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium.
In vivo analysis of DNase I hypersensitive sites in the human CFTR gene.
In vivo and in vitro correction of the mdx dystrophin gene nonsense mutation by short-fragment homologous replacement.
In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies.
In vivo cell-specific expression of the cystic fibrosis transmembrane conductance regulator.
In vivo evaluation of the safety of adenovirus-mediated transfer of the human cystic fibrosis transmembrane conductance regulator cDNA to the lung.
In vivo investigation of acidified pepsin exposure to porcine vocal fold epithelia.
In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis.
In vivo pharmacology and antidiarrheal efficacy of a thiazolidinone CFTR inhibitor in rodents.
In vivo transfer of the human cystic fibrosis transmembrane conductance regulator gene to the airway epithelium.
In-vitro evaluation of a ciprofloxacin- and ivacaftor-coated sinus stent against Pseudomonas aeruginosa biofilms.
In-vivo crystals reveal critical features of the interaction between CFTR and the PDZ2 domain of Na+/H+ exchange cofactor NHERF1.
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.
Incidence, etiology and prognosis of first-time acute pancreatitis in young patients: a population-based cohort study.
Incidence, Prevalence, Etiology, and Prognosis of First-Time Chronic Pancreatitis in Young Patients: A Nationwide Cohort Study.
Incomplete reprogramming after fusion of human multipotent stromal cells and bronchial epithelial cells.
Incomplete rescue of cystic fibrosis transmembrane conductance regulator deficient mice by the human CFTR cDNA.
Increase in intracellular Cl- concentration by cAMP- and Ca2+-dependent stimulation of M1 collecting duct cells.
Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice.
Increased apical Na+ permeability in cystic fibrosis is supported by a quantitative model of epithelial ion transport.
Increased arylsulfatase B activity in cystic fibrosis cells following correction of CFTR.
Increased chloride efflux in colchicine-resistant airway epithelial cell lines.
Increased circulating levels of plasma ATP in cystic fibrosis patients.
Increased contact time improves adenovirus-mediated CFTR gene transfer to nasal epithelium of CF mice.
Increased cystic fibrosis transmembrane conductance regulator (CFTR) expression in the human hydrosalpinx.
Increased diffusional mobility of CFTR at the plasma membrane after deletion of its C-terminal PDZ binding motif.
Increased expression of cystic fibrosis transmembrane conductance regulator in rat liver after common bile duct ligation.
Increased Expression of Plasma-Induced ABCC1 mRNA in Cystic Fibrosis.
Increased expression of sulfated gp300 and acinar tissue pathology in pancreas of CFTR(-/-) mice.
Increased expression of the secretory Na+-K+-2Cl- cotransporter with differentiation of a human intestinal cell line.
Increased extracellular vesicles mediate inflammatory signalling in cystic fibrosis.
Increased Fecal Calprotectin Is Associated with Worse Gastrointestinal Symptoms and Quality of Life Scores in Children with Cystic Fibrosis.
Increased Folding and Channel Activity of a Rare Cystic Fibrosis Mutant with CFTR Modulators.
Increased frequency of CFTR gene mutations identified in Indian infertile men with non-CBAVD obstructive azoospermia and spermatogenic failure.
Increased frequency of cystic fibrosis deltaF508 mutation in bronchiectasis associated with rheumatoid arthritis.
Increased frequency of cystic fibrosis transmembrane conductance regulator gene mutations in infertile males.
Increased functional cell surface expression of CFTR and DeltaF508-CFTR by the anthracycline doxorubicin.
Increased incidence of cystic fibrosis gene mutations in adults with disseminated bronchiectasis.
Increased intracellular Cl- concentration mediates Trichomonas vaginalis-induced inflammation in the human vaginal epithelium.
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.
Increased NF-?B Activity and Decreased Wnt/?-Catenin Signaling Mediate Reduced Osteoblast Differentiation and Function in ?F508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mice.
Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis.
Increased prevalence of mutations in the cystic fibrosis transmembrane conductance regulator in children with chronic rhinosinusitis.
Increased survival of CFTR knockout mice with an oral osmotic laxative.
Increased susceptibility of cftr-/- mice to LPS-induced lung remodeling.
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.
Increases in cytosolic Ca2+ induce dynamin- and calcineurin-dependent internalisation of CFTR.
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.
Indazole inhibition of cystic fibrosis transmembrane conductance regulator Cl(-) channels in rat epididymal epithelial cells.
Individualized medicine using intestinal responses to CFTR potentiators and correctors.
Induction of a cAMP-stimulated chloride secretion in regenerating poorly differentiated airway epithelial cells by adenovirus-mediated CFTR gene transfer.
Induction of bradykinin B1 receptors in rat colonic epithelium.
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.
Induction of expression of the cystic fibrosis transmembrane conductance regulator.
Induction of HSP70 promotes DeltaF508 CFTR trafficking.
Induction of multidrug resistance downregulates the expression of CFTR in colon epithelial cells.
Inefficient gene transfer by adenovirus vector to cystic fibrosis airway epithelia of mice and humans.
Infection by Toxoplasma gondii, a severe parasite in neonates and AIDS patients, causes impaired anion secretion in airway epithelia.
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.
Infertility in females with cystic fibrosis is multifactorial: evidence from mouse models.
Infertility in men with cystic fibrosis.
Inflammation and CFTR: might neutrophils be the key in cystic fibrosis?
Inflammation and its genesis in cystic fibrosis.
Inflammation in cystic fibrosis airways: relationship to increased bacterial adherence.
Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy.
Inflammation in cystic fibrosis: An update.
Inflammatory cytokines TNF-? and IL-17 enhance the efficacy of cystic fibrosis transmembrane conductance regulator modulators.
Inflammatory epithelial cytokines after in vitro respiratory syncytial viral infection are associated with reduced lung function.
Influence of cystic fibrosis transmembrane conductance regulator on gene expression in response to Pseudomonas aeruginosa infection of human bronchial epithelial cells.
Influence of molecular and chemical chaperones on protein folding.
Influence of salinity on the localization and expression of the CFTR chloride channel in the ionocytes of Dicentrarchus labrax during ontogeny.
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).
Influence of the cystic fibrosis transmembrane conductance regulator on expression of lipid metabolism-related genes in dendritic cells.
Influence of the duplication of CFTR exon 9 and its flanking sequences on diagnosis of cystic fibrosis mutations.
Influenza Matrix Protein 2 Alters CFTR Expression and Function through its Ion Channel Activity.
Influenza virus infection alters ion channel function of Airway and alveolar cells: Mechanisms and physiological sequelae.
Influenza virus M2 targets cystic fibrosis transmembrane conductance regulator for lysosomal degradation during viral infection.
Influenza-mediated reduction of lung epithelial ion channel activity leads to dysregulated pulmonary fluid homeostasis.
Infrared biomarkers of impaired cystic fibrosis transmembrane regulator protein biogenesis.
Inhaled Drug Therapy 2016: The Year in Review.
Inhaled interventions in cystic fibrosis: mucoactive and antibiotic therapies.
Inhaled phosphodiesterase type 5 inhibitors for cystic fibrosis: a new therapy for systemic disease?
Inhibiting endoplasmic reticulum (ER)-associated degradation of misfolded Yor1p does not permit ER export despite the presence of a diacidic sorting signal.
Inhibition by TNF-alpha and IL-4 of cationic lipid mediated gene transfer in cystic fibrosis tracheal gland cells.
Inhibition of Alk signaling promotes the induction of human salivary-gland-derived organoids.
Inhibition of amiloride-sensitive Na(+) absorption by activation of CFTR in mouse endometrial epithelium.
Inhibition of ATP-sensitive K+ channels by substituted benzo[c]quinolizinium CFTR activators.
Inhibition of ATPase, GTPase and adenylate kinase activities of the second nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator by genistein.
Inhibition of calpain blocks the phagosomal escape of Listeria monocytogenes.
Inhibition of cAMP- and Ca-dependent Cl- secretion by phorbol esters: inhibition of basolateral K+ channels.
Inhibition of CFTR channels by a peptide toxin of scorpion venom.
Inhibition of CFTR-mediated intestinal chloride secretion as potential therapy for bile acid diarrhea.
Inhibition of CFTR-mediated intestinal chloride secretion by a fungus-derived arthropsolide A: Mechanism of action and anti-diarrheal efficacy.
Inhibition of cystic fibrosis transmembrane conductance regulator by novel interaction with the metabolic sensor AMP-activated protein kinase.
Inhibition of cystic fibrosis transmembrane conductance regulator chloride channel currents by arachidonic acid.
Inhibition of ENaC by intracellular Cl- in an MDCK clone with high ENaC expression.
Inhibition of endocytosis suppresses the nitric oxide-dependent release of Cl- in retinal amacrine cells.
Inhibition of enterotoxin-induced porcine colonic secretion by diarylsulfonylureas in vitro.
Inhibition of epithelial Na+ currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator.
Inhibition of Heat-Stable Toxin-Induced Intestinal Salt and Water Secretion by a Novel Class of Guanylyl Cyclase C Inhibitors.
Inhibition of heterologously expressed cystic fibrosis transmembrane conductance regulator Cl- channels by non-sulphonylurea hypoglycaemic agents.
Inhibition of histone deacetylase 6 activity reduces cyst growth in polycystic kidney disease.
Inhibition of histone-deacetylase activity rescues inflammatory cystic fibrosis lung disease by modulating innate and adaptive immune responses.
Inhibition of IL-8 release from CFTR-deficient lung epithelial cells following pre-treatment with fenretinide.
Inhibition of matrix metalloproteinase MMP-2 activates chloride current in human airway epithelial cells.
Inhibition of Na+ /H+ exchanger isoform 3 improves gut fluidity and alkalinity in cystic fibrosis transmembrane conductance regulator-deficient and F508del mutant mice.
Inhibition of Na+,K+-ATPase by interferon gamma down-regulates intestinal epithelial transport and barrier function.
Inhibition of Na+-K+ pump and L-type Ca2+ channel by glibenclamide in Guinea pig ventricular myocytes.
Inhibition of neutral sodium absorption by a prostaglandin analogue in patients with cystic fibrosis.
Inhibition of NFkappaB by the natural product Withaferin A in cellular models of Cystic Fibrosis inflammation.
Inhibition of phosphatidylinositol 3-kinase does not alter forskolin-stimulated Cl(-) secretion by T84 cells.
Inhibition of the cystic fibrosis transmembrane conductance regulator by ATP-sensitive K+ channel regulators.
Inhibition of volume-regulated and calcium-activated chloride channels by the antimalarial mefloquine.
Inhibition of volume-regulated anion channels by expression of the cystic fibrosis transmembrane conductance regulator.
Inhibitors of ATP-binding cassette transporters suppress interleukin-12 p40 production and major histocompatibility complex II up-regulation in macrophages.
Inhibitory activities of curzerenone, curdione, furanodienone, curcumol and germacrone on Ca2+-activated chloride channels.
Inhibitory effects of glibenclamide on cystic fibrosis transmembrane regulator, swelling-activated, and Ca(2+)-activated Cl- channels in mammalian cardiac myocytes.
Inhibitory effects of tumor necrosis factor-alpha on cationic lipid-mediated gene delivery to airway cells in vitro.
Inhibitory regulation of cystic fibrosis transmembrane conductance regulator anion-transporting activities by Shank2.
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.
Innate immune activation and cystic fibrosis.
Innate immunity and chronic rhinosinusitis: What we have learned from animal models.
Innovating cystic fibrosis clinical trial designs in an era of successful standard of care therapies.
Innovative Therapies for Cystic Fibrosis: The Road from Treatment to Cure.
Insertion of an arginine residue into the transmembrane segments corrects protein misfolding.
Insight into cystic fibrosis by structural modelling of CFTR first nucleotide binding fold (NBF1).
Insights Into a Possible Influence on Gut Microbiota and Intestinal Barrier Function During Chronic Exposure of Mice to Imazalil.
Insights Into Patient Variability During Ivacaftor-Lumacaftor Therapy in Cystic Fibrosis.
Insights into the mechanisms underlying CFTR channel activity, the molecular basis for cystic fibrosis and strategies for therapy.
Insulin-like growth factor 1 (IGF-1) enhances the protein expression of CFTR.
Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis.
Integrative expression analysis identifies a novel interplay between CFTR and linc-SUMF1-2 that involves CF-associated gene dysregulation.
Interaction among variants in the SLC gene family (SLC6A14, SLC26A9, SLC11A1, and SLC9A3) and CFTR mutations with clinical markers of cystic fibrosis.
Interaction between 2 extracellular loops influences the activity of the cystic fibrosis transmembrane conductance regulator chloride channel.
Interaction between calcium-activated chloride channels and the cystic fibrosis transmembrane conductance regulator.
Interaction between CFTR and prestin (SLC26A5).
Interaction between cystic fibrosis transmembrane conductance regulator and outwardly rectified chloride channels.
Interaction between permeation and gating in a putative pore domain mutant in the cystic fibrosis transmembrane conductance regulator.
Interaction of intestinal and pancreatic transcription factors in the regulation of CFTR gene expression.
Interaction of nucleotides with membrane-associated cystic fibrosis transmembrane conductance regulator.
Interaction of syntaxins with epithelial ion channels.
Interaction of the protein phosphatase 2A with the regulatory domain of the cystic fibrosis transmembrane conductance regulator channel.
Interactions between ABCC4/MRP4 and ABCC7/CFTR in human airway epithelial cells in lung health and disease.
Interactions between impermeant blocking ions in the cystic fibrosis transmembrane conductance regulator chloride channel pore: evidence for anion-induced conformational changes.
Interactions between permeant and blocking anions inside the CFTR chloride channel pore.
Interdependency of beta-adrenergic receptors and CFTR in regulation of alveolar active Na+ transport.
Interdomain but not intermolecular interactions observed in CFTR channels.
Interest of colchicine for the treatment of cystic fibrosis patients. Preliminary report.
Interference with the formation of the epididymal microenvironment--a new strategy for male contraception?
Interferon regulatory factor 8 regulates expression of acid ceramidase and infection susceptibility in cystic fibrosis.
Interhelical hydrogen bonds in the CFTR membrane domain.
Interhelical packing in detergent micelles. Folding of a cystic fibrosis transmembrane conductance regulator construct.
Interleukin 2 modulates ion secretion and cell proliferation in cultured human small intestinal enterocytes.
Interleukin 8 secretion from monocytes of subjects heterozygous for the deltaF508 cystic fibrosis transmembrane conductance regulator gene mutation is altered.
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.
Interleukin-17A induces bicarbonate secretion in normal human bronchial epithelial cells.
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.
Interleukin-4 and interleukin-13 differentially regulate epithelial chloride secretion.
Interleukin-8 production by cystic fibrosis nasal epithelial cells after tumor necrosis factor-alpha and respiratory syncytial virus stimulation.
Intermolecular interaction between R domains of cystic fibrosis transmembrane conductance regulator.
Interplay between cystic fibrosis transmembrane regulator and gap junction channels made of connexins 45, 40, 32 and 50 expressed in oocytes.
Interplay between ER exit code and domain conformation in CFTR misprocessing and rescue.
Interplay between Inhibitory Ferric and Stimulatory Curcumin Regulates Phosphorylation-Dependent Human Cystic Fibrosis Transmembrane Conductance Regulator and ?F508 Activity.
Interregulation of proton-gated Na(+) channel 3 and cystic fibrosis transmembrane conductance regulator.
Interrelationship between the Na+/glucose cotransporter and CFTR in Caco-2 cells: relevance to cystic fibrosis.
Interruption of transmembrane signaling as a novel antisecretory strategy to treat enterotoxigenic diarrhea.
Interventions for preventing and managing advanced liver disease in cystic fibrosis.
Intestinal anion exchanger down-regulated in adenoma (DRA) is inhibited by intracellular calcium.
Intestinal bicarbonate secretion in cystic fibrosis mice.
Intestinal current measurement and nasal potential difference to make a diagnosis of cases with inconclusive CFTR genetics and sweat test.
Intestinal Enteroids Model Guanylate Cyclase C-Dependent Secretion Induced by Heat-Stable Enterotoxins.
Intestinal organoids and personalized medicine in cystic fibrosis: a successful patient-oriented research collaboration.
Intestinal pH and Gastrointestinal Transit Profiles in Cystic Fibrosis Patients Measured by Wireless Motility Capsule.
Intestinal physiology and pathology in gene-targeted mouse models of cystic fibrosis.
Intracellular CFTR: localization and function.
Intracellular cyclic AMP inhibits native and recombinant volume-regulated chloride channels from mammalian heart.
Intracellular cysteines of the cystic fibrosis transmembrane conductance regulator (CFTR) modulate channel gating.
Intracellular loop between transmembrane segments IV and V of cystic fibrosis transmembrane conductance regulator is involved in regulation of chloride channel conductance state.
Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins.
Intrathoracic nontuberculous mycobacterial infections in otherwise healthy children.
Intrinsic anion channel activity of the recombinant first nucleotide binding fold domain of the cystic fibrosis transmembrane regulator protein.
Introduction to PCR/OLA/SCS, a multiplex DNA test, and its application to cystic fibrosis.
Introduction to section II: omics in the biology of cystic fibrosis.
Introduction to section III: biochemical methods to study CFTR protein.
Intron-8 polythymidine sequence in Australasian individuals with CF mutations R117H and R117C.
Intronic enhancers coordinate epithelial-specific looping of the active CFTR locus.
Investigating Alternative Transport of Integral Plasma Membrane Proteins from the ER to the Golgi: Lessons from the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Investigating CFTR and KCa3.1 Protein/Protein Interactions.
Investigating hypothetical products from noncoding frames (HyPNoFs).
Involvement of AMP-activated Protein Kinase (AMPK) in Regulation of Cell Membrane Potential in a Gastric Cancer Cell Line.
Involvement of CFTR in oviductal HCO3- secretion and its effect on soluble adenylate cyclase-dependent early embryo development.
Involvement of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm.
Involvement of cystic fibrosis transmembrane conductance regulator (CFTR) in the pathogenesis of hydrosalpinx induced by Chlamydia trachomatis infection.
Involvement of cystic fibrosis transmembrane conductance regulator in infection-induced edema.
Involvement of cystic fibrosis transmembrane conductance regulator in mouse sperm capacitation.
Involvement of F1296 and N1303 of CFTR in induced-fit conformational change in response to ATP binding at NBD2.
Involvement of G protein betagamma-subunits in diverse signaling induced by G(i/o)-coupled receptors: study using the Xenopus oocyte expression system.
Involvement of hepatocyte nuclear factor 3 in endoderm differentiation of embryonic stem cells.
Involvement of ion channels in human eosinophil respiratory burst.
Involvement of phosphatidylinositol 3-kinase in cAMP- and cGMP-induced duodenal epithelial CFTR activation in mice.
Involvement of the anion exchanger SLC26A6 in prostaglandin E2- but not forskolin-stimulated duodenal HCO3- secretion.
Involvement of the Cdc42 Pathway in CFTR Post-Translational Turnover and in Its Plasma Membrane Stability in Airway Epithelial Cells.
Involvement of the cystic fibrosis transmembrane conductance regulator in the acidosis-induced efflux of ATP from rat skeletal muscle.
Involvement of the heterodimeric interface region of the nucleotide binding domain-2 (NBD2) in the CFTR quaternary structure and membrane stability.
Ion Channel Modulators in Cystic Fibrosis.
Ion channelopathies in functional GI disorders.
Ion channels as targets to treat cystic fibrosis lung disease.
Ion channels in the endometrium: regulation of endometrial receptivity and embryo implantation.
Ion Channels in The Pathogenesis of Endometriosis: A Cutting-Edge Point of View.
Ion transport across the jejunum in normal and cystic fibrosis mice.
Ion transport in the small intestine.
Ion transport mechanisms for smoke inhalation-injured airway epithelial barrier.
ION TRANSPORT MECHANISMS LINKED TO BICARBONATE SECRETION IN THE ESOPHAGEAL SUBMUCOSAL GLANDS.
Ionic mechanisms of cardiac cell swelling induced by blocking Na+/K+ pump as revealed by experiments and simulation.
Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells.
Iontophoretic beta-adrenergic stimulation of human sweat glands: possible assay for cystic fibrosis transmembrane conductance regulator activity in vivo.
iPSC-Derived Intestinal Organoids from Cystic Fibrosis Patients Acquire CFTR Activity upon TALEN-Mediated Repair of the p.F508del Mutation.
Irbit Mediates Synergy Between Ca2+ and cAMP Signaling Pathways During Epithelial Transport in Mice.
IRBIT: A regulator of ion channels and ion transporters.
IRE1 directs proteasomal and lysosomal degradation of misfolded rhodopsin.
Iron chelation as novel treatment for lung inflammation in cystic fibrosis.
Iron Homeostasis and Inflammatory Status in Mice Deficient for the Cystic Fibrosis Transmembrane Regulator.
Is acute recurrent pancreatitis a chronic disease?
Is acute recurrent pancreatitis in children a precursor of chronic pancreatitis? A long-term follow-up study of 93 cases.
Is CFTR an exchanger?: Regulation of HCO3-Transport and extracellular pH by CFTR.
Is congenital bilateral absence of vas deferens a primary form of cystic fibrosis? Analyses of the CFTR gene in 67 patients.
Is intracytoplasmic sperm injection itself an indication to perform preimplantation genetic diagnosis (PGD)? About PGD, invasive prenatal diagnosis and genetic sonography.
Is It Go or NO Go for S-Nitrosylation Modification-Based Therapies of Cystic Fibrosis Transmembrane Regulator Trafficking?
Is the ENaC Dysregulation in CF an Effect of Protein-Lipid Interaction in the Membranes?
Is there a role for stool metabolomics in cystic fibrosis?
Is vascular endothelial growth factor (VEGF) the main mediator in ovarian hyperstimulation syndrome (OHSS)?
Ischemia-induced enhancement of CFTR expression on the plasma membrane in neonatal rat ventricular myocytes.
Islet Function in the Pathogenesis of Cystic Fibrosis-Related Diabetes Mellitus.
Isobutylmethylxanthine fails to stimulate chloride secretion in cystic fibrosis airway epithelia.
Isoflavone genistein inhibits estrogen-induced chloride and bicarbonate secretory mechanisms in the uterus in rats.
Isoforms of SLC26A6 mediate anion transport and have functional PDZ interaction domains.
Isogenic cell models of cystic fibrosis-causing variants in natively expressing pulmonary epithelial cells.
Isolated elevated sweat chloride concentrations in the presence of the rare mutation S1455X: an extremely mild form of CFTR dysfunction.
Isolated idiopathic chronic pancreatitis associated with a compound heterozygosity for two mutations of the CFTR gene.
Isolation and characterization of a differentiation-dependent gene in the human colonic cell line HT29-18.
Isolation and characterization of rat cholangiocyte vesicles enriched in apical or basolateral plasma membrane domains.
Isolation and culture of bovine pancreatic duct epithelial cells.
Isolation of CFTR and TMEM16A inhibitors from Neorautanenia mitis (A. Rich) Verdcourt: Potential lead compounds for treatment of secretory diarrhea.
Isoprostane-mediated secretion from human airway epithelial cells.
Isorhamnetin Ameliorates Dry Eye Disease via CFTR Activation in Mice.
Isotype-specific activation of cystic fibrosis transmembrane conductance regulator-chloride channels by cGMP-dependent protein kinase II.
It's not all about inhaled treatment: challenges with oral therapy in paediatric respiratory medicine.
Ivacaftor and sinonasal pathology in a cystic fibrosis patient with genotype deltaF508/S1215N.
Ivacaftor for patients with cystic fibrosis.
Ivacaftor for the p.Ser549Arg (S549R) gating mutation - The Israeli experience.
Ivacaftor for the treatment of cystic fibrosis in children under six years of age.
Ivacaftor improves appearance of sinus disease on computerised tomography in cystic fibrosis patients with G551D mutation.
Ivacaftor improves rhinologic, psychologic, and sleep-related quality of life in G551D cystic fibrosis patients.
Ivacaftor in a young boy with the rare gating mutation S549R--use of lung clearance index to track progress: a case report.
Ivacaftor in People with Cystic Fibrosis and a 3849+10kb C?T or D1152H Residual Function Mutation.
Ivacaftor in severe cystic fibrosis lung disease and a G551D mutation.
Ivacaftor partially corrects airway inflammation in a humanized G551D rat.
IVACAFTOR restores FGF19 regulated bile acid homeostasis in cystic fibrosis patients with an S1251N or a G551D gating mutation.
Ivacaftor Therapy in CF Patients: Single Center Experience.
Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation.
Ivacaftor, a Cystic Fibrosis Transmembrane Conductance Regulator Potentiator, Enhances Ciprofloxacin Activity Against Pseudomonas aeruginosa.
Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis.
Ivacaftor: a novel gene-based therapeutic approach for cystic fibrosis.
Ivacaftor: a review of its use in patients with cystic fibrosis.
Ivacaftor: the first therapy acting on the primary cause of cystic fibrosis.
IVS8-5T Allele of CFTR is the Risk Factor in Chronic Pancreatitis, Especially in Idiopathic Chronic Pancreatitis.
Janus kinase 2 (JAK2) regulates prolactin-mediated chloride transport in mouse mammary epithelial cells through tyrosine phosphorylation of Na+-K+-2Cl- cotransporter.
K+ channels on resting duct cells from rat pancreas.
KCa3.1 potassium channels are critical for cAMP-dependent chloride secretion and cyst growth in autosomal-dominant polycystic kidney disease.
Keratin K18 increases cystic fibrosis transmembrane conductance regulator (CFTR) surface expression by binding to its C-terminal hydrophobic patch.
Kinin-stimulated chloride secretion in mouse colon requires the participation of CFTR chloride channels.
Knockdown of CFTR enhances sensitivity of prostate cancer cells to cisplatin via inhibition of autophagy.
Knockout mice reveal key roles for claudin 18 in alveolar barrier properties and fluid homeostasis.
Knockout mouse models for intestinal electrolyte transporters and regulatory PDZ adaptors: new insights into cystic fibrosis, secretory diarrhoea and fructose-induced hypertension.
KVLQT channels are inhibited by the K+ channel blocker 293B.
l-Methionine anti-biofilm activity against Pseudomonas aeruginosa is enhanced by the cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor.
L206W mutation of the cystic fibrosis gene, relatively frequent in French Canadians, is associated with atypical presentations of cystic fibrosis.
Laboratory and clinical studies in support of cystic fibrosis gene therapy using pCMV-CFTR-DOTAP.
Laboratory reporting on the clinical spectrum of CFTR p.Arg117His: Still room for improvement.
Lack of association of common cystic fibrosis transmembrane conductance regulator gene mutations with primary sclerosing cholangitis.
Lack of correlation between CFTR expression, CFTR Cl- currents, amiloride-sensitive Na+ conductance, and cystic fibrosis phenotype.
Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report.
Lack of cystic fibrosis transmembrane conductance regulator disrupts fetal airway development in pigs.
Lack of cystic fibrosis transmembrane regulator-type chloride current in pediatric human atrial myocytes.
Lack of effect of delta F508 mutation on aerobic capacity in patients with cystic fibrosis.
Lack of involvement of G proteins in the activation of cardiac CFTR Cl- current by genistein.
Lactobacillus casei protects dextran sodium sulfate- or rapamycin-induced colonic inflammation in the mouse.
Laminin 411 and 511 promote the cholangiocyte differentiation of human induced pluripotent stem cells.
Laser desorption mass spectrometry for point mutation detection.
Latest in cystic fibrosis.
Learning from each other: ABC transporter regulation by protein phosphorylation in plant and mammalian systems.
Lectin conjugates as potent, nonabsorbable CFTR inhibitors for reducing intestinal fluid secretion in cholera.
Left ventricular and aortic dysfunction in cystic fibrosis mice.
Lentiviral vectors for gene therapy of cystic fibrosis.
Lentiviral Vectors for the Treatment and Prevention of Cystic Fibrosis Lung Disease.
Lentiviral vectors transduce lung stem cells without disrupting plasticity.
Leporizines a-C: epithiodiketopiperazines isolated from an Aspergillus species.
Levamisole inhibits intestinal Cl- secretion via basolateral K+ channel blockade.
Levels of pro- and anti-inflammatory cytokines in cystic fibrosis patients with or without gingivitis.
Ligand binding to a remote site thermodynamically corrects the F508del mutation in the human cystic fibrosis transmembrane conductance regulator.
Ligand-induced dynamic changes in extended PDZ domains from NHERF1.
Light and alcohol evoked electro-oculograms in cystic fibrosis.
Limited restoration of cystic fibrosis lung epithelium in vivo with adult bone marrow-derived cells.
Linaclotide activates guanylate cyclase-C/cGMP/protein kinase-II-dependent trafficking of CFTR in the intestine.
Linaclotide Improves Gastrointestinal Transit in Cystic Fibrosis Mice by Inhibiting Sodium/Hydrogen Exchanger 3.
Linaclotide-a Novel Secretagogue in The Treatment of Irritable Bowel Syndrome with Constipation and Chronic Idiopathic Constipation.
Linaclotide: a new option for the treatment of irritable bowel syndrome with constipation and chronic idiopathic constipation in adults.
Linear-After-The-Exponential (LATE)-PCR: primer design criteria for high yields of specific single-stranded DNA and improved real-time detection.
Link between CFTR mutations and ABPA: a systematic review and meta-analysis.
Linkage analysis of candidate loci in autosomal dominant myotonia congenita.
Linkage disequilibrium between the M470V variant and the IVS8 polyT alleles of the CFTR gene in CBAVD.
Linkage to Gaucher mutations in the Ashkenazi population: effect of drift on decay of linkage disequilibrium and evidence for heterozygote selection.
Linoleic acid supplementation results in increased arachidonic acid and eicosanoid production in CF airway cells and in cftr-/- transgenic mice.
Lipid metabolism in cystic fibrosis.
Lipid Nanoparticle-Delivered Chemically Modified mRNA Restores Chloride Secretion in Cystic Fibrosis.
Lipid Peroxidation Drives Renal Cyst Growth In Vitro through Activation of TMEM16A.
Lipophilicity of the Cystic Fibrosis Drug, Ivacaftor (VX-770), and Its Destabilizing Effect on the Major CF-causing Mutation: F508del.
Liposomes loaded with bioactive lipids enhance antibacterial innate immunity irrespective of drug resistance.
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.
Liquid movement across the surface epithelium of large airways.
Liquid secretion properties of airway submucosal glands.
Liquid transport properties of porcine tracheal epithelium.
Listeria monocytogenes exploits cystic fibrosis transmembrane conductance regulator (CFTR) to escape the phagosome.
Listeria monocytogenes mediated CFTR transgene transfer to mammalian cells.
Lithocholic acid attenuates cAMP-dependent Cl- secretion in human colonic epithelial T84 cells.
Little Cigars are More Toxic than Cigarettes and Uniquely Change the Airway Gene and Protein Expression.
Liver cirrhosis and portal hypertension in cystic fibrosis.
Liver disease in cystic fibrosis.
Liver disease in pediatric patients with cystic fibrosis is associated with glutathione S-transferase P1 polymorphism.
Liver expression in cystic fibrosis could be modulated by genetic factors different from the cystic fibrosis transmembrane regulator genotype.
Liver X receptor ? regulates bile volume and the expression of aquaporins and cystic fibrosis transmembrane conductance regulator in the gallbladder.
LMTK2 Mediated Phosphorylation Regulates CFTR Endocytosis in Human Airway Epithelial Cells.
LncRNA and transcriptomic analysis of fetal membrane reveal potential targets involved in oligohydramnios.
Local modulation of Cystic Fibrosis Conductance Regulator: cytoskeleton and compartmentalised cAMP signalling.
Local regulation of cystic fibrosis transmembrane regulator and epithelial sodium channel in airway epithelium.
Localisation of wild-type and DeltaF508-CFTR in nasal epithelial cells.
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.
Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding.
Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia.
Localization of cystic fibrosis transmembrane conductance regulator in epithelial cells of nasal polyps and postoperative polypoid mucosae.
Localization of cystic fibrosis transmembrane conductance regulator mRNA in human fetal lung tissue by in situ hybridization.
Localization of cystic fibrosis transmembrane conductance regulator mRNA in the human gastrointestinal tract by in situ hybridization.
Localization of cystic fibrosis transmembrane conductance regulator signaling complexes in human salivary gland striated duct cells.
Localization of cystic fibrosis transmembrane conductance regulator to lipid rafts of epithelial cells is required for Pseudomonas aeruginosa-induced cellular activation.
Localization of sequences within the C-terminal domain of the cystic fibrosis transmembrane conductance regulator which impact maturation and stability.
Localization of the BiP molecular chaperone with respect to endoplasmic reticulum foci containing the cystic fibrosis transmembrane conductance regulator in yeast.
Localization of the cystic fibrosis transmembrane conductance regulator (Cftr) to mouse chromosome 6.
Localization of the cystic fibrosis transmembrane conductance regulator in airway secretory glands.
Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells.
Localization of the cystic fibrosis transmembrane conductance regulator in pancreas.
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.
Localization studies of rare missense mutations in cystic fibrosis transmembrane conductance regulator (CFTR) facilitate interpretation of genotype-phenotype relationships.
Localizing a gate in CFTR.
Locating a Plausible Binding Site for an Open Channel Blocker, GlyH-101, in the Pore of the Cystic Fibrosis Transmembrane Conductance Regulator.
Locating the anion-selectivity filter of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel.
Location of a common inhibitor binding site in the cytoplasmic vestibule of the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Location of a permeant anion binding site in the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Long Chain Polyunsaturated Fatty acids in Granulocytes, Mononuclear Cells and Red Blood Cells in Patients with Cystic Fibrosis: Relation to Liver Disease.
Long Non-coding RNA BGas Regulates the Cystic Fibrosis Transmembrane Conductance Regulator.
Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation.
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.
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.
Long-term cAMP activation of Na(+)-K(+)-2Cl- cotransporter activity in HT-29 human adenocarcinoma cells.
Long-term docosahexaenoic acid therapy in a congenic murine model of cystic fibrosis.
Long-Term Ivacaftor in People Aged 6 Years and Older with Cystic Fibrosis with Ivacaftor-Responsive Mutations.
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).
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.
Long-term therapeutic and reporter gene expression in lentiviral vector treated cystic fibrosis mice.
Long-wavelength iodide-sensitive fluorescent indicators for measurement of functional CFTR expression in cells.
Longevity and plasticity of CFTR provide an argument for noncanonical SNP organization in hominid DNA.
Longitudinal cystic fibrosis care.
Lonidamine and analogue AF2785 block the cyclic adenosine 3', 5'-monophosphate-activated chloride current and chloride secretion in the rat epididymis.
Loop diuretics are open-channel blockers of the cystic fibrosis transmembrane conductance regulator with distinct kinetics.
Loop Sequence Dictates the Secondary Structure of a Human Membrane Protein Hairpin.
Losartan reduces cigarette smoke-induced airway inflammation and mucus hypersecretion.
Loss of Ca-mediated ion transport during colitis correlates with reduced ion transport responses to a Ca-activated K channel opener.
Loss of CFTR Affects Biliary Epithelium Innate Immunity and Causes TLR4-NF-?B-Mediated Inflammatory Response in Mice.
Loss of Cftr function exacerbates the phenotype of Na+ hyperabsorption in murine airways.
Loss of cftr function leads to pancreatic destruction in larval zebrafish.
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.
Loss of cystic fibrosis transmembrane conductance regulator impairs lung endothelial cell barrier function and increases susceptibility to microvascular damage from cigarette smoke.
Loss of Cystic Fibrosis Transmembrane Regulator Impairs Intestinal Oxalate Secretion.
Loss of downregulated in adenoma (DRA) impairs mucosal HCO(3) (-) secretion in murine ileocolonic inflammation.
Loss of PKD1 and PKD2 share common effects on intracellular Ca2+ signaling.
Loss of Slc26a9 anion transporter alters intestinal electrolyte and HCO3 (-) transport and reduces survival in CFTR-deficient mice.
Loss of SLC9A3 decrease CFTR protein and causes obstructed azoospermia in mice.
Loss of the normal epicardial to endocardial gradient of cftr mRNA expression in the hypertrophied rabbit left ventricle.
Low abundance of sweat duct Cl- channel CFTR in both healthy and cystic fibrosis athletes with exceptionally salty sweat during exercise.
Low Beta-Adrenergic Sweat Responses in Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome Children.
Low sphingosine-1-phosphate impairs lung dendritic cells in cystic fibrosis.
Low temperature and chemical rescue affect molecular proximity of DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) and epithelial sodium channel (ENaC).
Lower Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Promotes the Proliferation and Migration of Endometrial Carcinoma.
Lower exhaled nitric oxide in infants with Cystic Fibrosis compared to healthy controls.
Lubiprostone activates non-CFTR-dependent respiratory epithelial chloride secretion in cystic fibrosis mice.
Lubiprostone Targets Prostanoid Signaling and Promotes Ion Transporter Trafficking, Mucus Exocytosis, and Contractility.
Lumacaftor alone and combined with ivacaftor: preclinical and clinical trial experience of F508del CFTR correction.
Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR.
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.
Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion.
Lumacaftor/ivacaftor therapy is associated with reduced hepatic steatosis in cystic fibrosis patients.
Lumacaftor/ivacaftor, a novel agent for the treatment of cystic fibrosis patients who are homozygous for the F580del CFTR mutation.
Lumacaftor/Ivacaftor: A Review in Cystic Fibrosis.
Luminal hydrochloric acid stimulates rapid transepithelial ion fluxes in rodent esophageal stratified squamous epithelium.
Luminally Acting Agents for Constipation Treatment: A Review Based on Literatures and Patents.
Luminally active, nonabsorbable CFTR inhibitors as potential therapy to reduce intestinal fluid loss in cholera.
Lung arginase expression and activity is increased in cystic fibrosis mouse models.
Lung disease associated with the IVS8 5T allele of the CFTR gene.
Lung edema clearance: 20 years of progress: invited review: alveolar edema fluid clearance in the injured lung.
Lung epithelial fluid transport and the resolution of pulmonary edema.
Lung function changes in relation to menstrual cycle in females with cystic fibrosis.
Lung gene therapy with highly compacted DNA nanoparticles that overcome the mucus barrier.
Lung infections associated with cystic fibrosis.
Lung symptoms in pseudohypoaldosteronism type 1 are associated with deficiency of the alpha-subunit of the epithelial sodium channel.
Luteolin reduces fluid hypersecretion by inhibiting TMEM16A in interleukin-4 treated Calu-3 airway epithelial cells.
Lymphocyte CFTR promotes epithelial bicarbonate secretion for bacterial killing.
Lysophosphatidic acid inhibits cholera toxin-induced secretory diarrhea through CFTR-dependent protein interactions.
Lysophosphatidylglycerol: a novel effective detergent for solubilizing and purifying the cystic fibrosis transmembrane conductance regulator.
Lysosomal Reacidification Ameliorates Vinyl Carbamate-Induced Toxicity and Disruption on Lysosomal pH.
Macromolecular complexes of cystic fibrosis transmembrane conductance regulator and its interacting partners.
Macrophage dysfunction in cystic fibrosis: Nature or nurture?
Magnetomotive optical coherence elastography for relating lung structure and function in Cystic Fibrosis.
Maintaining low Ca2+ level in the endoplasmic reticulum restores abnormal endogenous F508del-CFTR trafficking in airway epithelial cells.
Male partners of infertile couples with congenital unilateral absence of the vas deferens are mainly non-azoospermic.
Maltose transport through the inner membrane of E. coli.
Mammalian osmolytes and S-nitrosoglutathione promote Delta F508 cystic fibrosis transmembrane conductance regulator (CFTR) protein maturation and function.
Management of male infertility due to congenital bilateral absence of vas deferens should not ignore the diagnosis of cystic fibrosis.
Managing Fungal Infections in Cystic Fibrosis Patients: Challenges in Clinical Practice.
Manifestation of cystic fibrosis transmembrane regulator (CFTR) in hepatic ductal structures and renal tubules of female rats with experimental cholestasis of pregnancy.
Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis.
Mapping of cystic fibrosis transmembrane conductance regulator membrane topology by glycosylation site insertion.
Mapping of genetic factors influencing the weight of cystic fibrosis knockout mice.
Mapping the bovine homolog of the human cystic fibrosis gene.
Markedly elevated neonatal immunoreactive trypsinogen levels in the absence of cystic fibrosis gene mutations is not an indication for further testing.
Markers of early disease and prognosis in COPD.
Mashiningan Improves Opioid-Induced Constipation in Rats by Activating Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel.
MAST205 Competes with CAL for Binding to CFTR to Regulate CFTR-mediated Fluid Transport.
Maternal and fetal outcomes following elexacaftor-tezacaftor-ivacaftor use during pregnancy and lactation.
Matrine in association with FD?2 stimulates F508del?cystic fibrosis transmembrane conductance regulator activity in the presence of corrector VX809.
Matrine modulates HSC70 levels and rescues ?F508-CFTR.
Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2.
Maxi K+ channels co-localised with CFTR in the apical membrane of an exocrine gland acinus: possible involvement in secretion.
Maxillary sinusitis as a surrogate model for CF gene therapy clinical trials in patients with antrostomies.
Maximization of the rate of chloride conduction in the CFTR channel pore by ion-ion interactions.
Measurement of cystic fibrosis transmembrane conductance regulator activity using fluorescence spectrophotometry.
Measurement of ion transport function in rectal biopsies.
Measurement of Multi Ion Transport through Human Bronchial Epithelial Cell Line Provides an Insight into the Mechanism of Defective Water Transport in Cystic Fibrosis.
Mechanical Properties of Human Bronchial Epithelial Cells Expressing Wt- and Mutant CFTR.
Mechanical stress induces release of ATP from Ehrlich ascites tumor cells.
Mechanism and application of metformin in kidney diseases: An update.
Mechanism and cellular applications of a green fluorescent protein-based halide sensor.
Mechanism of activation of Xenopus CFTR by stimulation of PKC.
Mechanism of chloride permeation in the cystic fibrosis transmembrane conductance regulator chloride channel.
Mechanism of dysfunction of two nucleotide binding domain mutations in cystic fibrosis transmembrane conductance regulator that are associated with pancreatic sufficiency.
Mechanism of enhancement of DNA expression consequent to cointernalization of a replication-deficient adenovirus and unmodified plasmid DNA.
Mechanism of G551D-CFTR (cystic fibrosis transmembrane conductance regulator) potentiation by a high affinity ATP analog.
Mechanism of glibenclamide inhibition of cystic fibrosis transmembrane conductance regulator Cl- channels expressed in a murine cell line.
Mechanism of inhibition of cAMP-dependent epithelial chloride secretion by phorbol esters.
Mechanism of lonidamine inhibition of the CFTR chloride channel.
Mechanism of substance P-induced liquid secretion across bronchial epithelium.
Mechanism-based corrector combination restores ?F508-CFTR folding and function.
Mechanism-Based Personalized Medicine for Cystic Fibrosis by Suppressing Pseudo Exon Inclusion.
Mechanisms of attenuation of abdominal sepsis induced acute lung injury by ascorbic acid.
Mechanisms of bicarbonate secretion in the pancreatic duct.
Mechanisms of Ca2+-stimulated fluid secretion by porcine bronchial submucosal gland serous acinar cells.
Mechanisms of CFTR Folding at the Endoplasmic Reticulum.
Mechanisms of cystic fibrosis transmembrane conductance regulator activation by S-nitrosoglutathione.
Mechanisms of endothelial cell dysfunction in cystic fibrosis.
Mechanisms of fluid secretion by polycystic epithelia.
Mechanisms of guanylin action on water and ion absorption at different regions of seawater eel intestine.
Mechanisms of protein misfolding in conformational lung diseases.
Mechanisms of the inhibition of epithelial Na(+) channels by CFTR and purinergic stimulation.
Mechanisms of the noxious inflammatory cycle in cystic fibrosis.
Mechanistic analysis and significance of sphingomyelinase-mediated decreases in transepithelial CFTR currents in nHBEs.
Mechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic Fibrosis.
Mechanistic insight into control of CFTR by AMPK.
Mechanosensitive activation of CFTR by increased cell volume and hydrostatic pressure but not shear stress.
Mechanosensitive gating of CFTR.
Mechanosensitivity of wild-type and G551D cystic fibrosis transmembrane conductance regulator (CFTR) controls regulatory volume decrease in simple epithelia.
Medical devices; immunology and microbiology devices; classification of cystic fibrosis transmembrane conductance regulator gene mutation detection system. Final rule.
Medical interventions for chronic rhinosinusitis in cystic fibrosis.
Melatonin receptor potentiation of cyclic AMP and the cystic fibrosis transmembrane conductance regulator ion channel.
Membrane insertion, processing, and topology of cystic fibrosis transmembrane conductance regulator (CFTR) in microsomal membranes.
Membrane lateral diffusion and capture of CFTR within transient confinement zones.
Membrane protein stability can be compromised by detergent interactions with the extramembranous soluble domains.
Membrane targeting of cGMP-dependent protein kinase is required for cystic fibrosis transmembrane conductance regulator Cl- channel activation.
Membrane topology and glycosylation of the human multidrug resistance-associated protein.
Membrane topology distinguishes a subfamily of the ATP-binding cassette (ABC) transporters.
Membrane trafficking of the cystic fibrosis gene product, cystic fibrosis transmembrane conductance regulator, tagged with green fluorescent protein in madin-darby canine kidney cells.
Membrane-integration characteristics of two ABC transporters, CFTR and P-glycoprotein.
Membrane-specific regulation of Cl- channels by purinergic receptors in rat submandibular gland acinar and duct cells.
Mercury toxicity in the shark (Squalus acanthias) rectal gland: apical CFTR chloride channels are inhibited by mercuric chloride.
Mesenchymal Stem Cell exosome delivered Zinc Finger Protein activation of cystic fibrosis transmembrane conductance regulator.
Metabolic Reprograming of Cystic Fibrosis Macrophages via the IRE1? Arm of the Unfolded Protein Response Results in Exacerbated Inflammation.
Metabolic Signatures of Cystic Fibrosis Identified in Dried Blood Spots For Newborn Screening Without Carrier Identification.
Metabolomic profiling reveals biochemical pathways and biomarkers associated with pathogenesis in cystic fibrosis cells.
Metabolomic responses to lumacaftor/ivacaftor in cystic fibrosis.
Metal bridges illuminate transmembrane domain movements during gating of the cystic fibrosis transmembrane conductance regulator chloride channel.
Metformin slows liver cyst formation and fibrosis in experimental model of polycystic liver disease.
Metformin treatment of diabetes mellitus increases the risk for pancreatitis in patients bearing the CFTR-mutation S573C.
Methods for screening in cystic fibrosis.
Methods for the Use of Retroviral Vectors for Transfer of the CFTR Gene to Airway Epithelium.
Methods to monitor cell surface expression and endocytic trafficking of CFTR in polarized epithelial cells.
Methoxsalen stimulates electrogenic Cl- secretion in the mouse jejunum.
Methylation profile of the promoter CpG islands of 14 "drug-resistance" genes in hepatocellular carcinoma.
Methylomic correlates of autophagy activity in cystic fibrosis.
mGluR1 interacts with cystic fibrosis transmembrane conductance regulator and modulates the secretion of IL-10 in cystic fibrosis peripheral lymphocytes.
Micro-gallbladder: A rare surgical problem.
Micro-RNA-like effects of complete intronic sequences.
Microarray analysis reveals the inhibition of intestinal expression of nutrient transporters in piglets infected with porcine epidemic diarrhea virus.
Microbial clues lead to a diagnosis of cystic fibrosis in late adulthood.
Microbial-induced meprin ? cleavage in MUC2 mucin and a functional CFTR channel are required to release anchored small intestinal mucus.
Microelectrode measurements of the effects of basolateral adenosine in polarized human intestinal epithelial cells in culture.
MicroRNA Expression in Cystic Fibrosis Airway Epithelium.
MicroRNA profiling of cystic fibrosis intestinal disease in mice.
microRNA regulation of expression of the cystic fibrosis transmembrane conductance regulator gene.
MicroRNA-1246 regulates proliferation, invasion, and differentiation in human vascular smooth muscle cells by targeting cystic fibrosis transmembrane conductance regulator (CFTR).
MicroRNA-145, Cystic Fibrosis Transmembrane Conductance Regulator, and Transforming Growth Factor-?. An (Un)tangled Regulatory Web.
MicroRNA-9 downregulates the ANO1 chloride channel and contributes to cystic fibrosis lung pathology.
Microsatellite haplotypes associations with 5 CFTR mutations in "Grande Brière", an isolate located in southern Brittany.
Microtubule disruption inhibits AVT-stimulated Cl- secretion but not Na+ reabsorption in A6 cells.
Mid-trimester hyperechogenic bowel in a fetus of Japanese origin carrying a new mutation of CFTR gene (L548Q).
Mild course of cystic fibrosis associated with heterozygosity for infrequent mutations in the first nucleotide-binding fold of CFTR.
Mild cystic fibrosis in patients with the rare P5L CFTR mutation.
Milestones in cystic fibrosis.
Mineral content of calcified tissues in cystic fibrosis mice.
MiR-101 and miR-144 regulate the expression of the CFTR chloride channel in the lung.
miR-125b/NRF2/HO-1 axis is involved in protection against oxidative stress of cystic fibrosis: A pilot study.
miR-16 rescues F508del-CFTR function in native cystic fibrosis epithelial cells.
miR-17 overexpression in cystic fibrosis airway epithelial cells decreases interleukin-8 production.
miR-200b downregulates CFTR during hypoxia in human lung epithelial cells.
Misassembled mutant DeltaF508 CFTR in the distal secretory pathway alters cellular lipid trafficking.
Misfolding of the cystic fibrosis transmembrane conductance regulator and disease.
Mismatch-targeted transposition of Mu: a new strategy to map genetic polymorphism.
Misprocessing of the CFTR protein leads to mild cystic fibrosis phenotype.
Missense mutation within cystic fibrosis transmembrane conductance regulator (CFTR) gene is associated with selected parameters of the frozen-thawed sperm in Holstein-Friesian bulls.
Missense mutations in SLC26A8, encoding a sperm-specific activator of CFTR, are associated with human asthenozoospermia.
Missense mutations in the cystic fibrosis gene in adult patients with asthma.
Missense mutations in transmembrane domains of proteins: phenotypic propensity of polar residues for human disease.
Missense variants in CFTR nucleotide-binding domains predict quantitative phenotypes associated with cystic fibrosis disease severity.
Missense, nonsense, and neutral mutations define juxtaposed regulatory elements of splicing in cystic fibrosis transmembrane regulator exon 9.
Mitochondrial oxidative stress in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice.
Mitochondrial Stress Responses and "Mito-Inflammation" in Cystic Fibrosis.
Mobilization of intracellular Ca2+ and stimulation of cyclic AMP production by kappa opioid receptors expressed in Xenopus oocytes.
Model of mucociliary clearance in cystic fibrosis lungs.
Model of the cAMP activation of chloride transport by CFTR channel and the mechanism of potentiators.
Modeling Cystic Fibrosis Using Pluripotent Stem Cell-Derived Human Pancreatic Ductal Epithelial Cells.
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).
Modeling the Conformational Changes Underlying Channel Opening in CFTR.
Modelling Cl(-) homeostasis and volume regulation of the cardiac cell.
Modelling the restoration of wild-type dynamic behaviour in DeltaF508-CFTR NBD1 by 8-cyclopentyl-1,3-dipropylxanthine.
Modification of development by the CFTR gene in utero.
Modification of nasal epithelial potential differences of individuals with cystic fibrosis consequent to local administration of a normal CFTR cDNA adenovirus gene transfer vector.
Modification of transepithelial ion transport in human cultured bronchial epithelial cells by interferon-gamma.
Modifier genes in cystic fibrosis-related liver disease.
Modulating the folding of P-glycoprotein and cystic fibrosis transmembrane conductance regulator truncation mutants with pharmacological chaperones.
Modulation of CFTR chloride channels by calyculin A and genistein.
Modulation of CFTR gating by permeant ions.
Modulation of CFTR gene expression in HT-29 cells by extracellular hyperosmolarity.
Modulation of Chloride Channel Functions by the Plant Lignan Compounds Kobusin and Eudesmin.
Modulation of Cl- secretion by benzimidazolones. II. Coordinate regulation of apical GCl and basolateral GK.
Modulation of cystic fibrosis lung disease by variants in interleukin-8.
Modulation of cystic fibrosis transmembrane conductance regulator activity and genistein binding by cytosolic pH.
Modulation of cystic fibrosis transmembrane conductance regulator gene - expression by elevation of intracellular cyclic AMP.
Modulation of deltaF508 cystic fibrosis transmembrane regulator trafficking and function with 4-phenylbutyrate and flavonoids.
Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor.
Modulation of ENaC, CFTR, and iNOS expression in bronchial epithelial cells after stimulation with Staphylococcus epidermidis (94B080) and Staphylococcus aureus (90B083).
Modulation of epithelial sodium channel trafficking and function by sodium 4-phenylbutyrate in human nasal epithelial cells.
Modulation of Hepatic MRP3/ABCC3 by Xenobiotics and Pathophysiological Conditions: Role in Drug Pharmacokinetics.
Modulation of mature cystic fibrosis transmembrane regulator protein by the PDZ domain protein CAL.
Modulation of Polycystic Kidney Disease Severity by Phosphodiesterase 1 and 3 Subfamilies.
Modulation of protein kinase CK2 activity by fragments of CFTR encompassing F508 may reflect functional links with cystic fibrosis pathogenesis.
Modulation of sodium transport in alveolar epithelial cells by estradiol and progesterone.
Modulation of Treg function improves adenovirus vector-mediated gene expression in the airway.
Modulators of CFTR. Updates on clinical development and future directions.
Modulators of Ion Transport in Nasal Polyps: An in situ Measurement of Short-Circuit Current.
Modulators of the Cystic Fibrosis Transmembrane Conductance Regulator Protein.
Molecular analysis in Brazilian cystic fibrosis patients reveals five novel mutations.
Molecular analysis of 23 exons of the CFTR gene in Brazilian patients leads to the finding of rare cystic fibrosis mutations.
Molecular analysis of cystic fibrosis in the Hungarian population.
Molecular analysis of mutations and polymorphisms in the CFTR gene in male infertility.
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.
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.
Molecular analysis of the ovine cystic fibrosis transmembrane conductance regulator gene.
Molecular and cellular regulation of pancreatic duct cell function.
Molecular and Functional Analysis of the Large 5' Promoter Region of CFTR Gene Revealed Pathogenic Mutations in CF and CFTR-Related Disorders.
Molecular and functional characterization of CBAVD-causing mutations located in CFTR nucleotide-binding domains.
Molecular and functional characterization of the cystic fibrosis transmembrane conductance regulator from the Australian common brushtail possum, Trichosurus vulpecula.
Molecular and functional heterogeneity of cholangiocytes from rat liver after bile duct ligation.
Molecular assembly of cystic fibrosis transmembrane conductance regulator in plasma membrane.
Molecular Basis and Differentiation-Associated Alterations of Anion Secretion in Human Duodenal Enteroid Monolayers.
Molecular Basis for Fe(III)-Independent Curcumin Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Activity.
Molecular basis for the chloride channel activity of cystic fibrosis transmembrane conductance regulator and the consequences of disease-causing mutations.
Molecular basis of bicarbonate membrane transport in the male reproductive tract.
Molecular basis of cystic fibrosis in Lithuania: incomplete CFTR mutation detection by PCR-based screening protocols.
Molecular basis of cystic fibrosis in the Republic of Macedonia.
Molecular basis of hereditary pancreatitis.
Molecular basis of UG-rich RNA recognition by the human splicing factor TDP-43.
Molecular biology of cystic fibrosis.
Molecular Chaperones as Targets to Circumvent the CFTR Defect in 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.
Molecular characterization of the cystic fibrosis transmembrane conductance regulator gene in congenital absence of the vas deferens.
Molecular cloning and expression of a cyclic AMP-activated chloride conductance regulator: a novel ATP-binding cassette transporter.
Molecular cloning and sequence analysis of the murine cDNA for the cystic fibrosis transmembrane conductance regulator.
Molecular cloning, expression analysis, and chromosomal localization of human syntaxin 8 (STX8).
Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas.
Molecular determinants and role of an anion binding site in the external mouth of the CFTR chloride channel pore.
Molecular determinants of anion selectivity in the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Molecular determinants of Au(CN)(2)(-) binding and permeability within the cystic fibrosis transmembrane conductance regulator Cl(-) channel pore.
Molecular Diagnosis and Genetic Counseling of Cystic Fibrosis and Related Disorders: New Challenges.
Molecular diagnosis of congenital bilateral absence of the vas deferens: analyses of the CFTR gene in 64 French patients.
Molecular diagnosis of cystic fibrosis in Indian patients--a preliminary report.
Molecular diagnosis of cystic fibrosis.
Molecular Diagnostics of Cystic Fibrosis in Serbia: Our Approach to Meet the Diagnostic Challenges.
Molecular dissection of the butyrate action revealed the involvement of mitogen-activated protein kinase in cystic fibrosis transmembrane conductance regulator biogenesis.
Molecular Docking and QSAR Studies as Computational Tools Exploring the Rescue Ability of F508del CFTR Correctors.
Molecular domains in epithelial salt cellNaCl of crustacean salt gland (Artemia).
Molecular Dynamics Flexible Fitting Simulations Identify New Models of the Closed State of the Cystic Fibrosis Transmembrane Conductance Regulator Protein.
Molecular dynamics simulation study on the structural instability of the most common cystic fibrosis-associated mutant ?F508-CFTR.
Molecular genetic analysis of Turkish cystic fibrosis patients.
Molecular Genetics of Cystic Fibrosis Transmembrane Conductance Regulator: Genotype and Phenotype.
Molecular mechanism mediating enteric bacterial translocation after severe burn: the role of cystic fibrosis transmembrane conductance regulator.
Molecular mechanism of arachidonic acid inhibition of the CFTR chloride channel.
Molecular mechanisms of calcium-sensing receptor-mediated calcium signaling in the modulation of epithelial ion transport and bicarbonate secretion.
Molecular mechanisms of Cl- transport in fishes: New insights and their evolutionary context.
Molecular mechanisms of pancreatic stone formation in chronic pancreatitis.
Molecular modeling of the heterodimer of human CFTR's nucleotide-binding domains using a protein-protein docking approach.
Molecular modeling tools and approaches for CFTR and cystic fibrosis.
Molecular modelling and molecular dynamics of CFTR.
Molecular modelling approaches for cystic fibrosis transmembrane conductance regulator studies.
Molecular models of the open and closed states of the whole human CFTR protein.
Molecular Motors and Apical CFTR Traffic in Epithelia.
Molecular pathology of the CFTR locus in male infertility.
Molecular pathophysiology of cystic fibrosis based on the rescued knockout mouse model.
Molecular pharmacology of the CFTR Cl- channel.
Molecular physiology of anion channels.
Molecular physiology of CFTR Cl- channels in heart.
Molecular proximity of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel assessed by fluorescence resonance energy transfer.
Molecular receptive range variation among mouse odorant receptors for aliphatic carboxylic acids.
Molecular repair of a defective CFTR protein in cystic fibrosis.
Molecular screening of the CFTR gene in men with anomalies of the vas deferens: identification of three novel mutations.
Molecular species compositions of lung and pancreas phospholipids in the cftr(tm1HGU/tm1HGU) cystic fibrosis mouse.
Molecular structure of the ATP-bound, phosphorylated human CFTR.
Molecular Structure of the Human CFTR Ion Channel.
Molecular studies of CFTR interacting proteins.
Molecular study of (TG)m(T)n polymorphisms in Iranian males with congenital bilateral absence of the vas deferens.
Molecular temporal bone pathology: III. Genotyping of the deltaF508 deletion in the DNA of patients with cystic fibrosis.
Molecular understanding of chronic pancreatitis: a perspective on the future.
Monitoring early stage lung disease in cystic fibrosis.
MONNA, a Potent and Selective Blocker for Transmembrane Protein with Unknown Function 16/Anoctamin-1.
Monoclonal antibodies against MDR1 P-glycoprotein inhibit chloride conductance and label a 65-kDa protein in pancreatic zymogen granule membranes.
Monoclonal antibody against conductive chloride transport in pig ileal apical membrane vesicles.
Monocytes from cystic fibrosis patients are locked in an LPS tolerance state: down-regulation of TREM-1 as putative underlying mechanism.
Monogenic diabetes mellitus in cystic fibrosis.
Monomeric CFTR in plasma membranes in live cells revealed by single molecule fluorescence imaging.
Monovalent: Divalent Anion Selectivity in the CFTR Channel Pore.
Morbidity and mortality in carriers of the cystic fibrosis mutation CFTR Phe508del in the general population.
Morphologic effects of epithelial ion channels on the mouse uterus: differences between raloxifene analog (LY117018) and estradiol treatments.
Morphological and functional heterogeneity of the mouse intrahepatic biliary epithelium.
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.
Morphological, molecular, and functional heterogeneity of cholangiocytes from normal rat liver.
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin.
Motion--genetic testing is useful in the diagnosis of nonhereditary pancreatic conditions: arguments against the motion.
Motion--genetic testing is useful in the diagnosis of nonhereditary pancreatic conditions: arguments for the motion.
Motor dysfunction of the gut in cystic fibrosis.
Mouse cystic fibrosis transmembrane conductance regulator forms cAMP-PKA-regulated apical chloride channels in cortical collecting duct.
Mouse models of chronic lung infection with Pseudomonas aeruginosa: models for the study of cystic fibrosis.
Mouse organoid culture is a suitable model to study esophageal ion transport mechanisms.
mRNA transfection by a Xentry-protamine cell-penetrating peptide is enhanced by TLR antagonist E6446.
MRP class of human ATP binding cassette (ABC) transporters: historical background and new research directions.
MRP transporters as membrane machinery in the bradykinin-inducible export of ATP.
Mu 2 binding directs the cystic fibrosis transmembrane conductance regulator to the clathrin-mediated endocytic pathway.
mu-opioid receptor regulates CFTR coexpressed in Xenopus oocytes in a cAMP independent manner.
MUC4 expression is regulated by cystic fibrosis transmembrane conductance regulator in pancreatic adenocarcinoma cells via transcriptional and post-translational mechanisms.
Mucin glycosylation and sulphation in airway epithelial cells is not influenced by cystic fibrosis transmembrane conductance regulator expression.
Mucin overproduction in chronic inflammatory lung disease.
MUCLIN expression in the cystic fibrosis transmembrane conductance regulator knockout mouse.
Mucociliary clearance in cystic fibrosis knockout mice infected with Pseudomonas aeruginosa.
Mucociliary dysfunction in HIV and smoked substance abuse.
Mucous granule exocytosis and CFTR expression in gallbladder epithelium.
Mucus secretion by single tracheal submucosal glands from normal and cystic fibrosis transmembrane conductance regulator knockout mice.
Mucus, mucins, and cystic fibrosis.
Multi-dimensional clinical phenotyping of a national cohort of adult cystic fibrosis patients.
Multi-Ion mechanism for ion permeation and block in the cystic fibrosis transmembrane conductance regulator chloride channel.
Multi-ion pore behaviour in the CFTR chloride channel.
Multiallelic Synthetic Quality Control Material: Lessons Learned from the Cystic Fibrosis External Quality Assessment Scheme.
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.
Multicenter Intestinal Current Measurements in Rectal Biopsies from CF and Non-CF Subjects to Monitor CFTR Function.
Multidimensional clinical phenotyping of an adult cystic fibrosis patient population.
Multidrug resistance activity in human lymphocytes.
Multidrug resistance protein structure of Trypanosoma evansi isolated from buffaloes in Ngawi District, Indonesia: A bioinformatics analysis.
Multidrug resistance-associated proteins: Export pumps for conjugates with glutathione, glucuronate or sulfate.
Multifaceted structures and mechanisms of ABC transport systems in health and disease.
Multifactorial Genesis of Pancreatitis in Primary Hyperparathyroidism: Evidence for "Protective" (PRSS2) and "Destructive" (CTRC) Genetic Factors.
Multifunctional transporter models: lessons from the transport of water, sugars, and ring compounds by GLUTs.
Multiple endocytic signals in the C-terminal tail of the cystic fibrosis transmembrane conductance regulator.
Multiple inhibitory effects of Au(CN)(2-) ions on cystic fibrosis transmembrane conductance regulator Cl(-) channel currents.
Multiple intracellular pathways for regulation of chloride secretion in cultured pig tracheal submucosal gland cells.
Multiple Mechanisms Influence Regulation of the CFTR Gene Promoter.
Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gating.
Multiple mutation analysis of the cystic fibrosis gene in single cells.
Multiple mutations in highly conserved residues are found in mildly affected cystic fibrosis patients.
Multiple proteolytic systems, including the proteasome, contribute to CFTR processing.
Multiple transport functions of a red blood cell anion exchanger, tAE1: its role in cell volume regulation.
Multiplex allele-specific fluorescent PCR for haplotyping the IVS8 (TG)m(T)n locus in the CFTR gene.
Multiplex PCR of polymorphic markers flanking the CFTR gene; a general approach for preimplantation genetic diagnosis of 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.
Multiplexed genetic analysis using an expanded genetic alphabet.
Multiprobe Nuclear Imaging of the Cystic Fibrosis Lung as a Biomarker of Therapeutic Effect.
Multiscale approach to link red blood cell dynamics, shear viscosity, and ATP release.
Multitarget CFTR Modulators Endowed with Multiple Beneficial Side Effects for Cystic Fibrosis Patients: Toward a Simplified Therapeutic Approach †.
Mummichog gill and operculum exhibit functionally consistent claudin-10 paralog profiles and Claudin-10c hypersaline response.
Murine colonic mucosa hyperproliferation. I. Elevated CFTR expression and enhanced cAMP-dependent Cl(-) secretion.
Murine colonic mucosa hyperproliferation. II. PKC-beta activation and cPKC-mediated cellular CFTR overexpression.
Murine mCLCA6 is an integral apical membrane protein of non-goblet cell enterocytes and co-localizes with the cystic fibrosis transmembrane conductance regulator.
Muscarinic regulation of the cardiac CFTR Cl- current by quaternary ammonium compounds.
Mutant (delta F508) cystic fibrosis transmembrane conductance regulator Cl- channel is functional when retained in endoplasmic reticulum of mammalian cells.
Mutant CFTR Drives TWIST1 mediated epithelial-mesenchymal transition.
Mutant cycles at CFTR's non-canonical ATP-binding site support little interface separation during gating.
Mutant cystic fibrosis transmembrane conductance regulator inhibits acidification and apoptosis in C127 cells: possible relevance to 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.
Mutation analysis in 600 French cystic fibrosis patients.
Mutation analysis in the diagnosis of cystic fibrosis.
Mutation analysis of CFTR gene in 70 Iranian cystic fibrosis patients.
Mutation Analysis of Exons 10 and 17a of CFTR Gene in Patients with Cystic Fibrosis in Kermanshah Province, Western Iran.
Mutation analysis of PRSS1, SPINK1 and CFTR gene in patients with alcoholic and idiopathic chronic pancreatitis: A single center study.
Mutation Analysis of the CFTR Gene in 225 Children: Identification of Five Novel Severe and Seven Reported Severe Mutations.
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.
Mutation analysis of the cystic fibrosis transmembrane conductance regulator gene in Chinese congenital absence of vas deferens patients.
Mutation analysis of the cystic fibrosis transmembrane regulator gene in Native American populations of the southwest.
Mutation and new polymorphisms insight in introns 11 to 14a of CFTR gene of northern Iranian cystic fibrosis patients.
Mutation frequency of cystic fibrosis transmembrane regulator is not increased in oligozoospermic male candidates for intracytoplasmic sperm injection.
Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population.
Mutation nomenclature in practice: Findings and recommendations from the cystic fibrosis external quality assessment scheme.
Mutation of potential phosphorylation sites in the recombinant R domain of the cystic fibrosis transmembrane conductance regulator has significant effects on domain conformation.
Mutation of the aromatic amino acid interacting with adenine moiety of ATP to a polar residue alters the properties of multidrug resistance protein 1.
Mutation of Walker-A lysine 464 in cystic fibrosis transmembrane conductance regulator reveals functional interaction between its nucleotide-binding domains.
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.
Mutation-induced blocker permeability and multiion block of the CFTR chloride channel pore.
Mutation-specific potency and efficacy of cystic fibrosis transmembrane conductance regulator chloride channel potentiators.
Mutation-specific therapies and drug repositioning in cystic fibrosis.
Mutational analysis of CFTR in the Ecuadorian population using next-generation sequencing.
Mutational analysis of the Saccharomyces cerevisiae ATP-binding cassette transporter protein Ycf1p.
Mutational disruption of plasma membrane trafficking of Saccharomyces cerevisiae Yor1p, a homologue of mammalian multidrug resistance protein.
Mutational spectrum of cystic fibrosis in the Lebanese population.
Mutations and sequence variations detected in the cystic fibrosis transmembrane conductance regulator (CFTR) gene: a report from the Cystic Fibrosis Genetic Analysis Consortium.
Mutations at arginine 352 alter the pore architecture of CFTR.
Mutations at the signature sequence of CFTR create a Cd(2+)-gated chloride channel.
Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties.
Mutations in CFTR gene and clinical correlation in Argentine patients with congenital bilateral absence of the vas deferens.
Mutations in the amiloride-sensitive epithelial sodium channel in patients with cystic fibrosis-like disease.
Mutations in the amino terminus of the cystic fibrosis transmembrane conductance regulator enhance endocytosis.
Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens.
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) in Chinese patients with congenital bilateral absence of vas deferens.
Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials.
Mutations in the cystic fibrosis transmembrane regulator gene in patients with tropical calcific pancreatitis.
Mutations in the DNAH11 (axonemal heavy chain dynein type 11) gene cause one form of situs inversus totalis and most likely primary ciliary dyskinesia.
Mutations in the nucleotide binding domain 1 signature motif region rescue processing and functional defects of cystic fibrosis transmembrane conductance regulator delta f508.
Mutations in the putative pore-forming domain of CFTR do not change anion selectivity of the cAMP activated Cl- conductance.
Mutations in the SPINK1 gene in idiopathic pancreatitis Italian patients.
Mutations of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene Cause a Monocyte-selective Adhesion Deficiency.
Mutations of the CFTR gene in idiopathic pancreatic hyperenzymemia.
Mutations of the CFTR gene in Turkish patients with congenital bilateral absence of the vas deferens.
Mutations of the cystic fibrosis gene and intermediate sweat chloride levels in children.
Mutations of the cystic fibrosis gene in patients with chronic pancreatitis.
Mutations of the cystic fibrosis gene, but not cationic trypsinogen gene, are associated with recurrent or chronic idiopathic pancreatitis.
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).
Mutations that change the position of the putative gamma-phosphate linker in the nucleotide binding domains of CFTR alter channel gating.
Mutual Enhancement of Virulence by Enterotoxigenic and Enteropathogenic Escherichia coli.
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.
Myosin VI regulates endocytosis of the cystic fibrosis transmembrane conductance regulator.
N-Alpha-Acetyltransferases and Regulation of CFTR Expression.
N-glycans are direct determinants of CFTR folding and stability in secretory and endocytic membrane traffic.
N-terminal CFTR missense variants severely affect the behavior of the CFTR chloride channel.
N-terminal myristoylation is required for membrane localization of cGMP-dependent protein kinase type II.
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.
Na+ and Cl- conductances in airway epithelial cells: increased Na+ conductance in 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.
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.
Na+/HCO3- cotransport in normal and cystic fibrosis intestine.
Na+/K+/2Cl- cotransporter and CFTR gill expression after seawater transfer in smolts (0+) of different Atlantic salmon (Salmo salar) families
Na+:HCO(3-) cotransporters (NBC): cloning and characterization.
NaCl and fluid secretion by the intestine of the teleost Fundulus heteroclitus: involvement of CFTR.
Nanobody-based binding assay for the discovery of potent inhibitors of CFTR inhibitory factor (Cif).
Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating.
Nanomolar CFTR inhibition by pore-occluding divalent polyethylene glycol-malonic acid hydrazides.
Nanomolar Potency Pyrimido-pyrrolo-quinoxalinedione CFTR Inhibitor Reduces Cyst Size in a Polycystic Kidney Disease Model.
Nanomolar-potency 'co-potentiator' therapy for cystic fibrosis caused by a defined subset of minimal function CFTR mutants.
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.
Nanoparticles that deliver triplex-forming peptide nucleic acid molecules correct F508del CFTR in airway epithelium.
Naringenin Regulates CFTR Activation and Expression in Airway Epithelial Cells.
Nasal application of the cationic liposome DC-Chol:DOPE does not alter ion transport, lung function or bacterial growth.
Nasal potential difference in non-classic cystic fibrosis-long term follow up.
Nasal potential difference measurements in patients with atypical cystic fibrosis.
Nasal Potential Difference Test to Diagnose Cystic Fibrosis.
Nasal potential measurements on the nasal floor and under the inferior turbinate: Does it matter?
NDPK-A (but not NDPK-B) and AMPK alpha1 (but not AMPK alpha2) bind the cystic fibrosis transmembrane conductance regulator in epithelial cell membranes.
Nebulisation of receptor-targeted nanocomplexes for gene delivery to the airway epithelium.
Nedd4-2 Does Not Regulate wt-CFTR in Human Airway Epithelial Cells.
Negative chronotropic and inotropic effects of lubiprostone on iPS cell-derived cardiomyocytes via activation of CFTR.
Negative regulation of CFTR activity by extracellular ATP involves P2Y2 receptors in CFTR-expressing CHO cells.
Negative sweat test in hypertrypsinaemic infants with cystic fibrosis carrying rare CFTR mutations.
Neuraminidase inhibitors for the treatment of influenza infection in people with cystic fibrosis.
Neuropeptide regulation of secretion and inflammation in human airway gland serous cells.
Neutrophil elastase degrades cystic fibrosis transmembrane conductance regulator via calpains and disables channel function in vitro and in vivo.
Neutrophil extracellular trap (NET)-mediated killing of Pseudomonas aeruginosa: evidence of acquired resistance within the CF airway, independent of CFTR.
New advances in acute pancreatitis.
New and emerging targeted therapies for cystic fibrosis.
New and emerging therapies for pulmonary complications of cystic fibrosis.
New animal models of cystic fibrosis: what are they teaching us?
New concepts of the pathogenesis of cystic fibrosis lung disease.
New drug targets for cholera therapy.
New generation ENaC inhibitors detach cystic fibrosis airway mucus bundles via sodium/hydrogen exchanger inhibition.
New insights into autoimmune cholangitis through animal models.
New insights into cystic fibrosis: molecular switches that regulate CFTR.
New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation.
New Insights into the Binding Features of F508del CFTR Potentiators: A Molecular Docking, Pharmacophore Mapping and QSAR Analysis Approach.
New insights into the mechanisms underlying hydrosalpinx fluid formation and its adverse effect on IVF outcome.
New insights into the pathogenesis of cystic fibrosis: pivotal role of glutathione system dysfunction and implications for therapy.
New Model of Cystic Fibrosis Transmembrane Conductance Regulator Proposes Active Channel-like Conformation.
New models of the tracheal airway define the glandular contribution to airway surface fluid and electrolyte composition.
New pancreas cancers cell lines that represent distinct stages of ductal differentiation.
New paradigms of CFTR chloride channel regulation.
New pulmonary therapies directed at targets other than CFTR.
New pulmonary therapies for cystic fibrosis.
New Therapeutic Approaches to Modulate and Correct Cystic Fibrosis Transmembrane Conductance Regulator.
New therapies in cystic fibrosis.
New Therapies to Correct the Cystic Fibrosis Basic Defect.
New treatments targeting the basic defects in cystic fibrosis.
Newborn cystic fibrosis screening in southeastern Mexico: Birth prevalence and novel CFTR gene variants.
Newborn Screening for Cystic Fibrosis in California.
Newborn screening for cystic fibrosis in Wisconsin: comparison of biochemical and molecular methods.
Newborn screening methods for cystic fibrosis.
Next-Generation Sequencing for Molecular Diagnosis of Cystic Fibrosis in a Brazilian Cohort.
NF kappaB expression increases and CFTR and MUC1 expression decreases in the endometrium of infertile patients with hydrosalpinx: a comparative study.
NF-kappa B mediates up-regulation of CFTR gene expression in Calu-3 cells by interleukin-1beta.
NF-kappaB activation is involved in regulation of cystic fibrosis transmembrane conductance regulator (CFTR) by interleukin-1beta.
Nickel inhibits ?-1 adrenoceptor mediated activation of cardiac CFTR chloride channels.
Nicotine Induces Progressive Properties of Lung Adenocarcinoma A549 Cells by Inhibiting Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Expression and Plasma Membrane Localization.
Nine cystic fibrosis patients homozygous for the CFTR nonsense mutation R1162X have mild or moderate lung disease.
Nine mutations in the cystic fibrosis (CF) gene account for 80% of the CF chromosomes in French patients.
Nitric oxide activates chloride currents in human lung epithelial cells.
Nitric oxide has no beneficial effects on ion transport defects in cystic fibrosis human nasal epithelium.
Nitric oxide inhibits heterologous CFTR expression in polarized epithelial cells.
Nitric oxide synthase 1 as a potential modifier gene of decline in lung function in patients with cystic fibrosis.
NLR family pyrin domain containing 3 (NLRP3) and caspase 1 (CASP1) modulation by intracellular Cl- concentration.
NMR evidence for differential phosphorylation-dependent interactions in WT and DeltaF508 CFTR.
NMR measurements of the diffusional permeability of water in cultured colonic epithelial cancer cells.
NMR spectroscopy to study the dynamics and interactions of CFTR.
NMR structure of a type IVb pilin from Salmonella typhi and its assembly into pilus.
No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations.
No evidence for direct activation of the cystic fibrosis transmembrane conductance regulator by 8-cyclopentyl-1,3-dipropylxanthine.
No evidence for inhibition of ENaC through CFTR-mediated release of ATP.
No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis.
NO inhibits signal transduction pathway for ATP release from erythrocytes via its action on heterotrimeric G protein Gi.
Nobiletin Stimulates Chloride Secretion in Human Bronchial Epithelia via a cAMP/PKA-Dependent Pathway.
Nodular glomerulosclerosis in cystic fibrosis mimics diabetic nephropathy.
Non-classic cystic fibrosis associated with D1152H CFTR mutation.
Non-conventional trafficking of the cystic fibrosis transmembrane conductance regulator through the early secretory pathway.
Non-Genomic Effects of Fluticasone Propionate and Budesonide on Human Airway Anion Secretion.
Non-isocratic capillary electrophoresis for detection of DNA point mutations.
Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation.
Non-native interhelical hydrogen bonds in the cystic fibrosis transmembrane conductance regulator domain modulated by polar mutations.
Non-PCR methods for the analysis of CFTR transcripts.
Non-pore lining amino acid side chains influence anion selectivity of the human CFTR Cl- channel expressed in mammalian cell lines.
Non-radioactive detection of the most common mutations in the cystic fibrosis transmembrane conductance regulator gene by multiplex allele-specific polymerase chain reaction.
Non-specific activation of the epithelial sodium channel by the CFTR chloride channel.
Non-specificity of chloride channel blockers in rat cerebral arteries: block of the L-type calcium channel.
Non-viral gene transfer therapy for cystic fibrosis.
Non-viral mediated gene therapy in human cystic fibrosis airway epithelial cells recovers chloride channel functionality.
Nonequilibrium gating of CFTR on an equilibrium theme.
Nonintegral stoichiometry in CFTR gating revealed by a pore-lining mutation.
Nonisotopic detection of mutations using a modified single-strand conformation polymorphism analysis.
Nonradiometric ELISA-based quantitation and validation of polymerase chain reaction-amplified DNA, including detection of point mutations, without allele-specific amplification, or ligation.
Nonselective cation and Cl channels in luminal membrane of the marginal cell.
Nonsense mutation R1162X of the cystic fibrosis transmembrane conductance regulator gene does not reduce messenger RNA expression in nasal epithelial tissue.
Nonsteroidal anti-inflammatory drugs upregulate function of wild-type and mutant CFTR.
Nonstructural Protein NP1 of Human Bocavirus 1 Plays a Critical Role in the Expression of Viral Capsid Proteins.
Nontruncating SCN1A mutations associated with severe myoclonic epilepsy of infancy impair cell surface expression.
Nonvisualization of the Fetal Gallbladder: Can Levels of Gamma-Glutamyl Transpeptidase in Amniotic Fluid Predict Fetal Prognosis?
Normal and pathological erythrocytes studied by atomic force microscopy.
Normal CFTR Inhibits Epidermal Growth Factor Receptor-Dependent Pro-Inflammatory Chemokine Production in Human Airway Epithelial Cells.
Normal function of the cystic fibrosis conductance regulator protein can be associated with homozygous (Delta)F508 mutation.
Normal human pancreas cultures display functional ductal characteristics.
Normal mucus formation requires cAMP-dependent HCO3- secretion and Ca2+-mediated mucin exocytosis.
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.
Novel AAV-mediated gene delivery system corrects CFTR function in pigs.
Novel action of the chalcone isoliquiritigenin as a cystic fibrosis transmembrane conductance regulator (CFTR) inhibitor: potential therapy for cholera and polycystic kidney disease.
Novel Action of the Chalcone Isoliquiritigenin as a Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Inhibitor: Potential Therapy for Cholera and Polycystic Kidney Disease.
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.
Novel and recurrent rearrangements in the CFTR gene: clinical and laboratory implications for cystic fibrosis screening.
Novel ANO1 Inhibitor from Mallotus apelta Extract Exerts Anticancer Activity through Downregulation of ANO1.
Novel association of HLA-haplotypes with primary sclerosing cholangitis (PSC) in a southern European population.
Novel cause of hereditary obstructive azoospermia: a T2 allele in the CFTR gene.
Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquinolizinium lead compounds.
Novel cftr gene sequence variation in serbian patient with idiopathic disseminated bronchiectasis.
Novel CFTR Mutations in Two Iranian Families with Severe Cystic Fibrosis.
Novel CFTR Variants Identified during the First 3 Years of Cystic Fibrosis Newborn Screening in California.
Novel Correctors and Potentiators Enhance Functional Rescue of CFTR Nonsense Mutation Translational Readthrough.
Novel cystic fibrosis mutation associated with mild disease in Cypriot patients.
Novel de novo large deletion in cystic fibrosis transmembrane conductance regulator gene results in a severe cystic fibrosis phenotype.
Novel frameshift variant of the CFTR gene: S511Lfs*2 from phenotype to molecular predictions.
Novel GUCY2C variant causing familial diarrhea in a Mennonite kindred and a potential therapeutic approach.
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.
Novel Insight Into the Role of CFTR in Lacrimal Gland Duct Function in Mice.
Novel molecular approaches to cystic fibrosis gene therapy.
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.
Novel mutations and deletions in cystic fibrosis in a tertiary cystic fibrosis center in Istanbul.
Novel mutations and polymorphisms in the CFTR gene associated with three subtypes of congenital absence of vas deferens.
Novel opportunities for CFTR-targeting drug development using organoids.
Novel picolinamide-based cystic fibrosis transmembrane regulator modulators: evaluation of WO2013038373, WO2013038376, WO2013038381, WO2013038386 and WO2013038390.
Novel pore-lining residues in CFTR that govern permeation and open-channel block.
Novel regulation of cystic fibrosis transmembrane conductance regulator (CFTR) channel gating by external chloride.
Novel residues lining the CFTR chloride channel pore identified by functional modification of introduced cysteines.
Novel role for cystic fibrosis transmembrane conductance regulator in alveolar fluid clearance in lipopolysaccharide-induced acute lung injury in mice.
Novel role for pendrin in orchestrating bicarbonate secretion in cystic fibrosis transmembrane conductance regulator (CFTR)-expressing airway serous cells.
Novel role of cystic fibrosis transmembrane conductance regulator in maintaining adult mouse olfactory neuronal homeostasis.
Novel short chain fatty acids restore chloride secretion in cystic fibrosis.
Novel SIN-1 reactive intermediates modulate chloride secretion across murine airway cells.
Novel subunit composition of a renal epithelial KATP channel.
Novel, mechanism-based therapies for cystic fibrosis.
Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping.
Nucleoside triphosphate pentose ring impact on CFTR gating and hydrolysis.
Nucleosome mapping across the CFTR locus identifies novel regulatory factors.
Nucleosome occupancy reveals regulatory elements of the CFTR promoter.
Nucleotide occlusion in the human cystic fibrosis transmembrane conductance regulator. Different patterns in the two nucleotide binding domains.
Nucleotide release provides a mechanism for airway surface liquid homeostasis.
Nucleotide transport through the cystic fibrosis transmembrane conductance regulator.
Nucleotide-binding domain 1 of cystic fibrosis transmembrane conductance regulator production of a suitable protein for structural studies.
Nucleotide-binding Domains of Cystic Fibrosis Transmembrane Conductance Regulator, an ABC Transporter, Catalyze Adenylate Kinase Activity but Not ATP Hydrolysis.
Nucleotide-binding domains of human cystic fibrosis transmembrane conductance regulator: detailed sequence analysis and three-dimensional modeling of the heterodimer.
Nutritional effects on host response to lung infections with mucoid Pseudomonas aeruginosa in mice.
Nutritional status and eating disorders: neglected risks factor for nontuberculous mycobacterial lung disease?
Obesity and ethnicity alter gene expression in skin.
Obliterative Portal Venopathy Without Cirrhosis Is Prevalent in Pediatric Cystic Fibrosis Liver Disease With Portal Hypertension.
Ocular Surface Potential Difference Measured in Human Subjects to Study Ocular Surface Ion Transport.
Omission of exon 12 in cystic fibrosis transmembrane conductance regulator (CFTR) gene transcripts.
On the Corner of Models and Cure: Gene Editing in Cystic Fibrosis.
On the discovery and development of CFTR chloride channel activators.
On the interactions between nucleotide binding domains and membrane spanning domains in cystic fibrosis transmembrane regulator: A molecular dynamic study.
On the mechanism of CFTR inhibition by a thiazolidinone derivative.
On the mechanism of genistein-induced activation of protein kinase A-dependent Cl- conductance in cardiac myocytes.
On the origin of asymmetric interactions between permeant anions and the cystic fibrosis transmembrane conductance regulator chloride channel pore.
On the relationship between anion binding and chloride conductance in the CFTR anion channel.
On the structural organization of the intracellular domains of CFTR.
One-step affinity isolation of recombinant protein using the baculovirus/insect cell expression system.
Ontogeny of CLCN3 chloride channel gene expression in human pulmonary epithelium.
Ontogeny of guanylin-immunoreactive cells in rat salivary glands.
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.
Opiates inhibit ion conductances elicited by cell swelling and cAMP in cultured cells.
Opportunistic infections in lung disease: Pseudomonas infections in cystic fibrosis.
Optical imaging of Ca2+-evoked fluid secretion by murine nasal submucosal gland serous acinar cells.
Optical imaging of Cl- permeabilities in normal and CFTR-expressing mouse L cells.
Optimal Complement-Mediated Phagocytosis of Pseudomonas aeruginosa by Monocytes Is Cystic Fibrosis Transmembrane Conductance Regulator-Dependent.
Optimal correction of distinct CFTR folding mutants in rectal cystic fibrosis organoids.
Optimisation of real-time quantitative RT-PCR for the evaluation of non-viral mediated gene transfer to the airways.
Optimization of a Yellow fluorescent protein-based iodide influx high-throughput screening assay for cystic fibrosis transmembrane conductance regulator (CFTR) modulators.
Optimization of cationic liposome-mediated gene transfer to human bronchial epithelial cells expressing wild-type or abnormal cystic fibrosis transmembrane conductance regulator (CFTR).
Optimization of hCFTR lung expression in mice using DNA nanoparticles.
Optimization of rAAV-mediated Expression for Large Transgenes Using a Synthetic Promoter and Tandem Array Enhancers.
Optimization of the degenerated interfacial ATP binding site improves the function of diseases related mutant cystic fibrosis transmembrane conductance regulator (CFTR) channels.
Optimized detection of DNA point mutations by double gradient denaturing gradient gel electrophoresis.
Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation.
Organ-specific over-sulfation of glycosaminoglycans and altered extracellular matrix in a mouse model of cystic fibrosis.
Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator.
Oridonin: A small molecule inhibitor of cystic fibrosis transmembrane conductance regulator (CFTR) isolated from traditional Chinese medicine.
Origin and evolution of the cystic fibrosis transmembrane regulator protein R domain.
Origin and utility of the reverse dot-blot.
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.
Orphan missense mutations in the cystic fibrosis transmembrane conductance regulator a three-step biological approach to establishing a correlation between genotype and phenotype.
Osmoregulation in the Plotosidae Catfish: Role of the Salt Secreting Dendritic Organ.
Osteoblast CFTR inactivation reduces differentiation and osteoprotegerin expression in a mouse model of cystic fibrosis-related bone disease.
Osteoclastogenesis and sphingosine-1-phosphate secretion from human osteoclast precursor monocytes are modulated by the cystic fibrosis transmembrane conductance regulator.
Osteopenia in Cftr-deltaF508 mice.
OTX2 regulates CFTR expression during endoderm differentiation and occupies 3' cis-regulatory elements.
Ouabain Mimics Low Temperature Rescue of F508del-CFTR in Cystic Fibrosis Epithelial Cells.
Ouabain Regulates CFTR-Mediated Anion Secretion and Na,K-ATPase Transport in ADPKD Cells.
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.
Outcomes of a cystic fibrosis carrier testing clinic for couples.
Outwardly rectifying chloride channels and CF: a divorce and remarriage.
Overcoming Immunological Challenges to Helper-Dependent Adenoviral Vector-Mediated Long-Term CFTR Expression in Mouse Airways.
Overexpression of cystic fibrosis transmembrane conductance regulator (CFTR) is associated with human cervical cancer malignancy, progression and prognosis.
Overexpression of R domain eliminates cAMP-stimulated Cl- secretion in 9/HTEo- cells in culture.
Overexpression of the cystic fibrosis transmembrane conductance regulator in NIH 3T3 cells lowers membrane potential and intracellular pH and confers a multidrug resistance phenotype.
Overexpression, purification, and function of first nucleotide-binding fold of cystic fibrosis transmembrane conductance regulator.
Overproduction of the CFTR R domain leads to increased levels of asialoGM1 and increased Pseudomonas aeruginosa binding by epithelial cells.
Ovine male genital duct epithelial cells differentiate in vitro and express functional CFTR and ENaC.
Oxidant and antioxidant modulation of chloride channels expressed in human retinal pigment epithelium.
Oxidant stress stimulates anion secretion from the human airway epithelial cell line Calu-3: implications for cystic fibrosis lung disease.
Oxidative stress and antioxidant therapy in cystic fibrosis.
Oxidative stress causes IL8 promoter hyperacetylation in cystic fibrosis airway cell models.
Oxidative stress regulates CFTR gene expression in human airway epithelial cells through a distal antioxidant response element.
Oxygen-glucose deprivation induces ATP release via maxi-anion channels in astrocytes.
Ozone stress down-regulates the expression of cystic fibrosis transmembrane conductance regulator in human bronchial epithelial cells.
P-glycoprotein expression increases ATP release in respiratory cystic fibrosis cells.
P-glycoprotein inhibition by glibenclamide and related compounds.
P-glycoprotein. ATP hydrolysis by the N-terminal nucleotide-binding domain.
p.F508del in a heterogeneous cystic fibrosis population from Minas Gerais, Brazil.
p.Ser1235Arg should no longer be considered as a cystic fibrosis mutation: results from a large collaborative study.
P2Y purinergic receptor regulation of CFTR chloride channels in mouse cardiac myocytes.
P2Y6 receptor mediates colonic NaCl secretion via differential activation of cAMP-mediated transport.
Pancreatic and Islet Remodeling in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Knockout Ferrets.
Pancreatic complications in children with cystic fibrosis.
Pancreatic ductal bicarbonate secretion: past, present and future.
Pancreatic insufficiency and pulmonary disease in German and Slavic cystic fibrosis patients with the R347P mutation.
Pancreatic intraepithelial neoplasia is associated with chronic pancreatitis due to serine protease inhibitor kazal type 1 and cystic fibrosis transmembrane conductance regulator mutations.
Pancreatitis and cystic fibrosis gene mutations.
Pancreatitis in A Patient with Cystic Fibrosis Taking Ivacaftor.
Pancreatitis risk in primary hyperparathyroidism: relation to mutations in the SPINK1 trypsin inhibitor (N34S) and the cystic fibrosis gene.
Pancreatitis severity in mice with impaired CFTR function but pancreatic sufficiency is mediated via ductal and inflammatory cells-Not acinar cells.
Paracellular bicarbonate flux across human cystic fibrosis airway epithelia tempers changes in airway surface liquid pH.
Paracellular Permeability of Bronchial Epithelium is Controlled by CFTR.
Paracellular transport through healthy and cystic fibrosis bronchial epithelial cell lines--do we have a proper model?
Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells.
Parallel single nucleotide polymorphism genotyping by surface invasive cleavage with universal detection.
Paranasal sinus size is decreased in CFTR heterozygotes with chronic rhinosinusitis.
Parasympathetic innervation regulates tubulogenesis in the developing salivary gland.
Parathyroid hormone increases CFTR expression and function in Caco-2 intestinal epithelial cells.
Partial correction of endogenous DeltaF508 CFTR in human cystic fibrosis airway epithelia by spliceosome-mediated RNA trans-splicing.
Partial purification of the cystic fibrosis transmembrane conductance regulator.
Partial purification of the pig kidney cystic fibrosis transmembrane regulator protein.
Participation of cAMP in a signal-transduction pathway relating erythrocyte deformation to ATP release.
Participation of the Cl-/HCO(3)- exchangers SLC26A3 and SLC26A6, the Cl- channel CFTR, and the regulatory factor SLC9A3R1 in mouse sperm capacitation.
Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator.
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.
Patch-clamp studies of cystic fibrosis transmembrane conductance regulator chloride channel.
Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases.
Pathogen acquisition in patients with cystic fibrosis receiving ivacaftor or lumacaftor/ivacaftor.
Pathogen and autoantigen homologous regions within the cystic fibrosis transmembrane conductance regulator (CFTR) protein suggest an autoimmune treatable component of cystic fibrosis.
Pathogen-induced secretory diarrhea and its prevention.
Pathogenic role of ADGRG2 in CBAVD patients replicated in Chinese population.
Pathology of gastrointestinal organs in a porcine model of cystic fibrosis.
Pathophysiologic consequences following inhibition of a CFTR-dependent developmental cascade in the lung.
Pathophysiological mechanisms of diarrhea caused by the Vibrio cholerae O1 El Tor variant: an in vivo study in mice.
Pathophysiology and management of pulmonary infections in cystic fibrosis.
Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota.
Pathophysiology of Hyperechogenic Bowel in Congenitally Human Cytomegalovirus Infected Fetuses.
Pathway prediction by bioinformatic analysis of the untranslated regions of the CFTR mRNA.
Pathways in the Pathophysiology of Coronavirus 19 Lung Disease Accessible to Prevention and Treatment.
Patient and family issues regarding genetic testing for cystic fibrosis: a review of prenatal carrier testing and newborn screening.
Patient personalized translational tools in cystic fibrosis to transform data from bench to bed-side and back.
Patient perspectives following initiation of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis and advanced lung disease.
Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey.
Patients with mutations in Gsalpha have reduced activation of a downstream target in epithelial tissues due to haploinsufficiency.
Paucity of CFTR current but modest CFTR immunoreactivity in non-diseased human ventricle.
PCR bias toward the wild-type k-ras and p53 sequences: implications for PCR detection of mutations and cancer diagnosis.
PCR from single cells for preimplantation diagnosis.
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.
PDZ domain interaction controls the endocytic recycling of the cystic fibrosis transmembrane conductance regulator.
PDZ Structure and Implication in Selective Drug Design against Cystic Fibrosis.
PDZ-binding motifs are unable to ensure correct polarized protein distribution in the absence of additional localization signals.
PDZK1 Upregulation in Estrogen-Related Hyperpigmentation in Melasma.
Pediatric population with cystic fibrosis in the centre of Portugal: Candidates for new therapies.
Pendrin stimulates a chloride absorption pathway to increase CFTR-mediated chloride secretion from Cystic Fibrosis airway epithelia.
Peptide binding consensus of the NHE-RF-PDZ1 domain matches the C-terminal sequence of cystic fibrosis transmembrane conductance regulator (CFTR).
Peptide inhibitors of chloride channels for treating secretory diarrhea.
Peptide microarray analysis of substrate specificity of the transmembrane Ser/Thr kinase KPI-2 reveals reactivity with cystic fibrosis transmembrane conductance regulator and phosphorylase.
Peptide Nucleic Acids for MicroRNA Targeting.
Peptide surface display and secretion using two LPXTG-containing surface proteins from Lactobacillus fermentum BR11.
Perinuclear positioning of the inactive human cystic fibrosis gene depends on CTCF, A-type lamins and an active histone deacetylase.
Peripheral localization of the epithelial sodium channel in the apical membrane of bronchial epithelial cells.
Peripheral Protein Quality Control as a Novel Drug Target for CFTR Stabilizer.
Peripheral protein quality control removes unfolded CFTR from the plasma membrane.
Permeability of wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride channels to polyatomic anions.
Permeation through the CFTR chloride channel.
Permissiveness of human biliary epithelial cells to infection by hepatitis C virus.
Persistent interactions between the two transmembrane clusters dictate the targeting and functional assembly of adenylyl cyclase.
Persistent osteopenia in adult cystic fibrosis transmembrane conductance regulator-deficient mice.
Personalized medicine for cystic fibrosis: establishing human model systems.
Personalized Medicine in Cystic Fibrosis: Dawning of a New Era.
Perspectives on gene therapy for cystic fibrosis airway disease.
Perspectives on mucus properties and formation--lessons from the biochemical world.
Perturbation of the pore of the cystic fibrosis transmembrane conductance regulator (CFTR) inhibits its atpase activity.
PGE2 Regulation of CFTR Activity and Air Surface Liquid Volume Requires Gap Junctional Communication.
pH stat studies on bicarbonate secretion in the isolated mouse ileum.
Pharmaceuticals targeting nonsense mutations in genetic diseases: progress in development.
Pharmacogenomics of the cystic fibrosis transmembrane conductance regulator (CFTR) and the cystic fibrosis drug CPX using genome microarray analysis.
Pharmacokinetic and Drug-Drug Interaction Profiles of the Combination of Tezacaftor/Ivacaftor.
Pharmacokinetics and safety of cavosonstat (N91115) in healthy and cystic fibrosis adults homozygous for F508DEL-CFTR.
Pharmacologic modulation of salt and water in the airway epithelium in cystic fibrosis.
Pharmacologic therapy for stop mutations: how much CFTR activity is enough?
Pharmacological analysis of CFTR variants of cystic fibrosis using stem cell-derived organoids.
Pharmacological approaches for targeting cystic fibrosis nonsense mutations.
Pharmacological approaches to correcting the ion transport defect in cystic fibrosis.
Pharmacological correction of a defect in PPAR-gamma signaling ameliorates disease severity in Cftr-deficient mice.
Pharmacological Correction of Cystic Fibrosis: Molecular Mechanisms at the Plasma Membrane to Augment Mutant CFTR Function.
Pharmacological Inhibition of the Ubiquitin Ligase RNF5 Rescues F508del-CFTR in Cystic Fibrosis Airway Epithelia.
Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia.
Pharmacological Rescue of Conditionally Reprogrammed Cystic Fibrosis Bronchial Epithelial Cells.
Pharmacological Rescue of Ductal CFTR Rescue Pancreatic and Salivary Glands Acinar Cells and Tissue Function in Mouse Models of Autoimmune Diseases.
Pharmacological reversal of renal cysts from secretion to absorption suggests a potential therapeutic strategy for managing autosomal dominant polycystic kidney disease.
Pharmacological therapy for cystic fibrosis: from bench to bedside.
Pharmacological treatment of the basic defect in cystic fibrosis.
Pharmacological treatment of the biochemical defect in cystic fibrosis airways.
Pharmacological treatment of the ion transport defect in cystic fibrosis.
Pharmacology of CFTR chloride channel activity.
Pharmacotherapy of the ion transport defect in cystic fibrosis.
Pharmacotherapy of the ion transport defect in cystic fibrosis: role of purinergic receptor agonists and other potential therapeutics.
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.
Phasing DNA Markers Using Digital PCR.
PhenCode: connecting ENCODE data with mutations and phenotype.
Phenotype and genotype of two Taiwanese cystic fibrosis siblings and a survey of delta F508 in East Asians.
Phenotype of CF and the effects of possible modifier genes.
Phenotype-optimized sequence ensembles substantially improve prediction of disease-causing mutation in cystic fibrosis.
Phenotypes of California CF Newborn Screen-Positive Children with CFTR 5T Allele by TG Repeat Length.
Phenotypic and Genetic Characterization of Carbapenemase and ESBLs Producing Gram-negative Bacteria (GNB) Isolated from Patients with Cystic Fibrosis (CF) in Tehran Hospitals.
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.
Phenotypic expression of genotype-phenotype correlation in cystic fibrosis patients carrying the 852del22 mutation.
Phenotypic plasticity in gene expression and physiological response in red drum Sciaenops ocellatus exposed to a long-term freshwater environment.
Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia.
Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTATM.
Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function.
Phenylglycine and sulfonamide correctors of defective delta F508 and G551D cystic fibrosis transmembrane conductance regulator chloride-channel gating.
Phenylhydrazones as Correctors of a Mutant Cystic Fibrosis Transmembrane Conductance Regulator.
Phenylquinoxalinone CFTR activator as potential prosecretory therapy for constipation.
Phloxine B interacts with the cystic fibrosis transmembrane conductance regulator at multiple sites to modulate channel activity.
Phosphatase inhibitors activate normal and defective CFTR chloride channels.
Phosphate stimulates CFTR Cl- channels.
Phosphatidic acid metabolism regulates the intracellular trafficking and retrotranslocation of CFTR.
Phosphatidylcholine Passes by Paracellular Transport to the Apical Side of the Polarized Biliary Tumor Cell Line Mz-ChA-1.
Phosphodiesterase 4D forms a cAMP diffusion barrier at the apical membrane of the airway epithelium.
Phosphodiesterase-4 Inhibitors for Non-COPD Respiratory Diseases.
Phosphorylated and ubiquitinated TDP-43 pathological inclusions in ALS and FTLD-U are recapitulated in SH-SY5Y cells.
Phosphorylated C/EBP? influences a complex network involving YY1 and USF2 in lung epithelial cells.
Phosphorylation by cAMP-dependent protein kinase causes a conformational change in the R domain of the cystic fibrosis transmembrane conductance regulator.
Phosphorylation by protein kinase C is required for acute activation of cystic fibrosis transmembrane conductance regulator by protein kinase A.
Phosphorylation of CFTR by PKA promotes binding of the regulatory domain.
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.
Phosphorylation of P-glycoprotein by PKA and PKC modulates swelling-activated Cl- currents.
Phosphorylation of PDZ1 domain attenuates NHERF-1 binding to cellular targets.
Phosphorylation of protein kinase C sites in NBD1 and the R domain control CFTR channel activation by PKA.
Phosphorylation of S955 at the protein kinase A consensus promotes maturation of the alpha subunit of the colonic H+,K+ -ATPase.
Phosphorylation of the Chaperone-Like HspB5 Rescues Trafficking and Function of F508del-CFTR.
Phosphorylation of the cystic fibrosis transmembrane conductance regulator.
Phosphorylation of vimentin is an intermediate step in protein kinase C-mediated glycoconjugate secretion.
Phosphorylation-dependent 14-3-3 protein interactions regulate CFTR biogenesis.
Phosphorylation-dependent block of cystic fibrosis transmembrane conductance regulator chloride channel by exogenous R domain protein.
Phosphorylation-dependent modulation of CFTR macromolecular signalling complex activity by cigarette smoke condensate in airway epithelia.
Phosphorylation-induced conformational changes of cystic fibrosis transmembrane conductance regulator monitored by attenuated total reflection-Fourier transform IR spectroscopy and fluorescence spectroscopy.
Phosphorylation-regulated Cl- channel in CHO cells stably expressing the cystic fibrosis gene.
Phylogenetic analysis of cystic fibrosis transmembrane conductance regulator gene in mammalian species argues for the development of a rabbit model for cystic fibrosis.
Physiologic and molecular aspects of the Na+:HCO3- cotransporter in health and disease processes.
Physiological and molecular mechanisms of osmoregulatory plasticity in killifish after seawater transfer.
Physiological and Pathological Functions of Cl- Channels in Chondrocytes.
Physiological and Pathophysiological Relevance of the Anion Transporter Slc26a9 in Multiple Organs.
Physiological concept for a blood based CFTR test.
Physiological evidence for a P2Y receptor responsive to diadenosine polyphosphates in human lung via Ca(2+) release studies in bronchial epithelial cells.
Physiological impact of abnormal lipoxin A? production on cystic fibrosis airway epithelium and therapeutic potential.
Physiological implications of the regulation of vacuolar H+-ATPase by chloride ions.
Physiological modulation of CFTR activity by AMP-activated protein kinase in polarized T84 cells.
Physiological regulation of ATP release at the apical surface of human airway epithelia.
Physiological study of pulmonary involvement in adults with cystic fibrosis through simulated modeling of different clinical scenarios.
Physiologically Based Pharmacokinetic Modeling of CFTR Modulation in People with Cystic Fibrosis Transitioning from Mono or Dual Regimens to Triple-Combination Elexacaftor/Tezacaftor/Ivacaftor.
Physiology of apoptosis.
Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth.
PIKfyve upregulates CFTR activity.
Pinacidil and levamisole prevent glutamate-induced death of hippocampal neuronal cells through reducing ROS production.
Pitfall in the use of genotype analysis as the sole diagnostic criterion for cystic fibrosis.
Pitfalls in the immunohistochemical localization of the cystic fibrosis transmembrane conductance regulator in paraffin embedded sweat glands.
Pitfalls in the interpretation of CFTR variants in the context of incidental findings.
PKA holoenzyme is functionally coupled to CFTR by AKAPs.
PKA Mediates Constitutive Activation of CFTR in Human Sweat Duct.
PKA phosphorylation potentiates CFTR gating by relieving auto-inhibition on the stimulatory C terminus of the regulatory domain.
PKC regulation of cardiac CFTR Cl- channel function in guinea pig ventricular myocytes.
PKC-sensitive Cl- channels associated with ciliary epithelial homologue of pICln.
Plasma Levels of the Bioactive Sphingolipid Metabolite S1P in Adult Cystic Fibrosis Patients: Potential Target for Immunonutrition?
Plasma membrane Ca2+ ATPase isoform 2b interacts preferentially with Na+/H+ exchanger regulatory factor 2 in apical plasma membranes.
Plasma membrane protein clusters appear in CFTR-expressing Xenopus laevis oocytes after cAMP stimulation.
Plasma membrane recycling in CFTR-expressing CHO cells.
Plasma membrane-localized TMEM16 proteins are indispensable for expression of CFTR.
Plasmodium falciparum-activated chloride channels are defective in erythrocytes from cystic fibrosis patients.
Platelet activation in cystic fibrosis.
Platelets: inflammatory effector cells in the conflagration of cystic fibrosis lung disease.
Pleiotropic effect of vitamin D in cystic fibrosis.
Plumbagin Prevents Secretory Diarrhea by Inhibiting CaCC and CFTR Channel Activities.
Pluripotent Stem Cell Platforms for Drug Discovery.
Pluripotent stem cells for disease modeling and drug screening: new perspectives for treatment of cystic fibrosis?
Polar residues in membrane domains of proteins: molecular basis for helix-helix association in a mutant CFTR transmembrane segment.
Polarization of specific tropomyosin isoforms in gastrointestinal epithelial cells and their impact on CFTR at the apical surface.
Polarization-dependent apical membrane CFTR targeting underlies cAMP-stimulated Cl- secretion in epithelial cells.
Polarized AAVR expression determines infectivity by AAV gene therapy vectors.
Polarized distribution of HCO3- transport in human normal and cystic fibrosis nasal epithelia.
Polarized distribution of key membrane transport proteins in the rat submandibular gland.
Polarized expression of cAMP-activated chloride channels in isolated epithelial cells.
Polarized expression of cystic fibrosis transmembrane conductance regulator and associated epithelial proteins during the regeneration of human airway surface epithelium in three-dimensional culture.
Polarized signaling via purinoceptors in normal and cystic fibrosis airway epithelia.
Poly thymidine polymorphism and cystic fibrosis in a non-Caucasian population.
Polymorphic expression of multidrug resistance mRNA in lung parenchyma of nonpregnant and pregnant rats: a comparison to cystic fibrosis mRNA expression.
Polymorphic markers suggest a gene flow of CFTR gene from Sub-Saharan/Arabian and Mediterranean to Brazilian Population.
Polymorphism of cystic fibrosis gene in Japanese patients with chronic pancreatitis.
Polymorphisms in the glutathione pathway modulate cystic fibrosis severity: a cross-sectional study.
Polytopic membrane protein folding and assembly in vitro and in vivo.
Polyvariant mutant CFTR genes in patients with chronic pancreatitis.
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.
Population screening of F508del (DeltaF508), the most frequent mutation in the CFTR gene associated with cystic fibrosis in Argentina.
Population-specific screening by mutation analysis for diseases frequent in Ashkenazi Jews.
Porcine nasal epithelial cultures for studies of cystic fibrosis sinusitis.
Pore dilation increases the bicarbonate permeability of CFTR, ANO1, and glycine receptor anion channels.
Positional dependence of non-native polar mutations on folding of CFTR helical hairpins.
Positional effects of premature termination codons on the biochemical and biophysical properties of CFTR.
Positioning of extracellular loop 1 affects pore gating of the cystic fibrosis transmembrane conductance regulator.
Positive charges at the intracellular mouth of the pore regulate anion conduction in the CFTR chloride channel.
Positive epistasis between disease-causing missense mutations and silent polymorphism with effect on mRNA translation velocity.
Positive neonatal screening for cystic fibrosis in neonates with renal failure.
Post-Transcriptional Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Expression and Function by MicroRNAs.
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.
Postnatal decrease of cystic fibrosis transmembrane conductance regulator gene expression in nasal epithelium of healthy newborn infants.
Potent inhibition of the CFTR chloride channel by suramin.
Potent s-cis-locked bithiazole correctors of DeltaF508 cystic fibrosis transmembrane conductance regulator cellular processing for cystic fibrosis therapy.
Potent stimulation and inhibition of the CFTR Cl(-) current by phloxine B.
Potent, Metabolically Stable Benzopyrimido-pyrrolo-oxazine-dione (BPO) CFTR Inhibitors for Polycystic Kidney Disease.
Potential genetic modifiers of the cystic fibrosis intestinal inflammatory phenotype on mouse chromosomes 1, 9, and 10.
Potential of helper-dependent Adenoviral vectors in CRISPR-cas9-mediated lung gene therapy.
Potential of Intestinal Current Measurement for Personalized Treatment of Patients with Cystic Fibrosis.
Potential role for the common cystic fibrosis DeltaF508 mutation in Crohn's disease.
Potential role of abnormal ion transport in the pathogenesis of chronic sinusitis.
Potential role of CFTR in bisphenol A-induced malignant transformation of prostate cells via mitochondrial apoptosis.
Potential role of the "NADPH oxidases" (NOX/DUOX) family in cystic fibrosis.
Potentially lethal cystic fibrosis gene variant in the orangutan.
Potentiation of cystic fibrosis transmembrane conductance regulator (CFTR) Cl- currents by the chemical solvent tetrahydrofuran.
Potentiation of disease-associated CFTR mutants by hydrolyzable ATP analogs.
Potentiation of effect of PKA stimulation of Xenopus CFTR by activation of PKC: role of NBD2.
Potentiation of mutant CFTR Cl- channel currents by the naturally occurring stilbene compound resveratrol.
Potentiation of the cystic fibrosis transmembrane conductance regulator by VX-770 involves stabilization of the pre-hydrolytic, O1 state.
Potentiation of the cystic fibrosis transmembrane conductance regulator Cl- channel by ivacaftor is temperature-independent.
Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis.
Potentiators and Correctors in Paediatric Cystic Fibrosis Patients: A Narrative Review.
Potentiators of Defective ?F508-CFTR Gating that Do Not Interfere with Corrector Action.
PPAR{gamma} Agonists Inhibit Vasopressin Mediated Anion Transport in the MDCK-C7 Cell Line.
Pre-clinical evaluation of three non-viral gene transfer agents for cystic fibrosis after aerosol delivery to the ovine lung.
Precision toxicology shows that troxerutin alleviates ochratoxin A-induced renal lipotoxicity.
Preclinical evidence that sildenafil and vardenafil activate chloride transport in 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.
Predicting the risk of cystic fibrosis with echogenic fetal bowel and one cystic fibrosis mutation.
Prediction of the antigenic sites of the cystic fibrosis transmembrane conductance regulator protein by molecular modelling.
Predictive value of cystic fibrosis transmembrane conductance regulator (CFTR) in the diagnosis of gastric cancer.
Predictors of deterioration of lung function in cystic fibrosis.
Pregnancy in cystic fibrosis: Review of the literature and expert recommendations.
Preimplantation genetic diagnosis for cystic fibrosis: a case report.
Preimplantation genetic diagnosis of cystic fibrosis (delta F508).
Preliminary evidence for cell membrane amelioration in children with cystic fibrosis by 5-MTHF and vitamin B12 supplementation: a single arm trial.
Preliminary study of haplotypes linked to the rare cystic fibrosis E1104X mutation.
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.
Prenatal diagnosis of cystic fibrosis in different European populations: application of denaturing gradient gel electrophoresis.
Prenatal screening for cystic fibrosis: past, present and future.
Preoperative serum methylation signature as prognostic tool after curative hepatectomy in patients with hepatocellular carcinoma.
Prerenal azotemia from excessive sweating in an adult with a cystic fibrosis gene mutation.
Presence of CFTR in the conjunctival epithelium.
Presence of MRP8 and MRP14 in pancreatic cell lines: differential expression and localization in CFPAC-1 cells.
Prevalence of ?F508 mutation in the cystic fibrosis transmembrane conductance regulator gene among cystic fibrosis patients from a Brazilian referral center.
Prevalence of CBAVD in azoospermic men carrying pathogenic CFTR mutations - Evaluated in a cohort of 639 non-vasectomized azoospermic men.
Prevalence of meconium ileus marks the severity of mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene.
Prevention and treatment of secretory diarrhea by the lysophosphatidic acid analog Rx100.
Prevention of chronic Pseudomonas aeruginosa infection in people with cystic fibrosis.
Prevention of drug-related complications in cystic fibrosis.
Prevention of toxin-induced intestinal ion and fluid secretion by a small-molecule CFTR inhibitor.
Previously unknown role for the ubiquitin ligase Ubr1 in endoplasmic reticulum-associated protein degradation.
Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function.
Primary sclerosing cholangitis is associated with abnormalities in CFTR.
Pro-resolving lipid mediator Resolvin D1 serves as a marker of lung disease in cystic fibrosis.
Pro-Secretory Activity and Pharmacology in Rabbits of an Aminophenyl-1,3,5-Triazine CFTR Activator for Dry Eye Disorders.
Probing an open CFTR pore with organic anion blockers.
Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant.
Probing the basic defect in cystic fibrosis.
Probing the structural and functional domains of the CFTR chloride channel.
Probiotics and Commensals Reverse TNF-alpha- and IFN-gamma-Induced Dysfunction in Human Intestinal Epithelial Cells.
Processing and function of CFTR-DeltaF508 are species-dependent.
Processing Mutations Disrupt Interactions between the Nucleotide Binding and Transmembrane Domains of P-glycoprotein and the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Processing mutations located throughout the human multidrug resistance P-glycoprotein disrupt interactions between the nucleotide binding domains.
Processing of CFTR bearing the P574H mutation differs from wild-type and deltaF508-CFTR.
Processing of CFTR: traversing the cellular maze--how much CFTR needs to go through to avoid cystic fibrosis?
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive.
Production and characterisation of monoclonal and polyclonal antibodies to different regions of the cystic fibrosis transmembrane conductance regulator (CFTR): detection of immunologically related proteins.
Production and effects of alpha-melanocyte-stimulating hormone during acute lung injury.
Production of CFTR-?F508 Rabbits.
Production of cystic fibrosis transmembrane conductance regulator in the milk of transgenic mice.
Profile of cystic fibrosis in a single referral center in Egypt.
Profile of tezacaftor/ivacaftor combination and its potential in the treatment of cystic fibrosis.
Progress in therapies for cystic fibrosis.
Progress toward generating a ferret model of cystic fibrosis by somatic cell nuclear transfer.
Progression of early structural lung disease in young children with cystic fibrosis assessed using CT.
Proinflammatory Cytokine Secretion is Suppressed by TMEM16A or CFTR Channel Activity in Human Cystic Fibrosis Bronchial Epithelia.
Proinflammatory cytokines inhibit secretion in rat bile duct epithelium.
Proliferation, not apoptosis, alters epithelial cell migration in small intestine of CFTR null mice.
Prolonged administration of secretin to normal rats increases biliary proliferation and secretin-induced ductal secretory activity.
Prolonged treatment of cells with genistein modulates the expression and function of the cystic fibrosis transmembrane conductance regulator.
Promoter architecture modulates CFTR exon 9 skipping.
Promoter hypermethylation of the CFTR gene and clinical/pathological features associated with non-small cell lung cancer.
Promoter hypermethylation of the CFTR gene as a novel diagnostic and prognostic marker of breast cancer.
Properties and actions of heat-stable enterotoxin of Escherichia coli.
Properties of a Cl(-)-conductive pathway(s) in microsomes from rat kidney inner medulla. Involvement of cystic fibrosis transmembrane regulator protein.
Properties of cAMP-dependent and Ca(2+)-dependent whole cell Cl- conductances in rat epididymal cells.
Properties of CFTR activated by the xanthine derivative X-33 in human airway Calu-3 cells.
Properties of chloride-conductive pathways in rat kidney cortical and outer-medulla brush-border membranes--inhibition by anti-(cystic fibrosis transmembrane regulator) mAbs.
Properties of the cAMP-activated C1- current in choroid plexus epithelial cells isolated from the rat.
Propofol inhibits carbachol-induced chloride secretion by directly targeting the basolateral K(+) channel in rat ileum epithelium.
Proportion of cystic fibrosis gene mutations not detected by routine testing in men with obstructive azoospermia.
Prospective analysis of cystic fibrosis transmembrane regulator mutations in adults with bronchiectasis or pulmonary nontuberculous mycobacterial infection.
Prospective and parallel assessments of cystic fibrosis newborn screening protocols in the Czech Republic: IRT/DNA/IRT versus IRT/PAP and IRT/PAP/DNA.
Prospects for gene therapy for cystic fibrosis.
Prostacyclin receptor-mediated ATP release from erythrocytes requires the voltage-dependent anion channel.
Prostaglandin E2 induces chloride secretion through crosstalk between cAMP and calcium signaling in mouse inner medullary collecting duct cells.
Prostaglandin E2 stimulates anion and fluid secretion triggered by lipopolysaccharide in rat vaginal epithelium.
Prostaglandin F2alpha stimulates CFTR activity by PKA- and PKC-dependent phosphorylation.
Prostanoids secreted by alveolar macrophages enhance ionic currents in swine tracheal submucosal gland cells.
Protease-activated receptor 2 mediates mucus secretion in the airway submucosal gland.
Protease-activated receptor-1 stimulates Ca(2+)-dependent Cl(-) secretion in human intestinal epithelial cells.
Proteases and antiproteases in cystic fibrosis: pathogenetic considerations and therapeutic strategies.
Proteasome-dependent pharmacological rescue of cystic fibrosis transmembrane conductance regulator revealed by mutation of glycine 622.
Protection against bronchial asthma by CFTR delta F508 mutation: a heterozygote advantage in cystic fibrosis.
Protection against oxidative stress in beta thalassemia/hemoglobin E erythrocytes by inhibitors of glutathione efflux transporters.
Protection of Cftr knockout mice from acute lung infection by a helper-dependent adenoviral vector expressing Cftr in airway epithelia.
Protective role of cardiac CFTR activation upon early reperfusion against myocardial infarction.
Protein folding. Translational tuning optimizes nascent protein folding in cells.
Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites.
Protein kinase A associates with cystic fibrosis transmembrane conductance regulator via an interaction with ezrin.
Protein kinase C activates chloride conductance in C127 cells stably expressing the cystic fibrosis gene.
Protein kinase C and Ca2+ activation of mucin secretion in airway goblet cells.
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.
Protein kinase C mediates down-regulation of cystic fibrosis transmembrane conductance regulator levels in epithelial cells.
Protein kinase C phosphorylation disrupts Na+/H+ exchanger regulatory factor 1 autoinhibition and promotes cystic fibrosis transmembrane conductance regulator macromolecular assembly.
Protein kinase C regulates the magnitude and stability of CFTR currents in pancreatic duct cells.
Protein kinase CK2, cystic fibrosis transmembrane conductance regulator, and the deltaF508 mutation: F508 deletion disrupts a kinase-binding site.
Protein kinase-independent activation of CFTR by phosphatidylinositol phosphates.
Protein phosphatase 1 coordinates CFTR-dependent airway epithelial HCO3 (-) secretion by reciprocal regulation of apical and basolateral membrane Cl(-) -HCO3 (-) exchangers.
Protein phosphatase 2C dephosphorylates and inactivates cystic fibrosis transmembrane conductance regulator.
Protein trafficking rates assessed by quantum dot quenching with bromocresol green.
Proteinuria in cystic fibrosis: a possible correlation between genotype and renal phenotype.
Proteome of the porosome complex in human airway epithelia: interaction with the cystic fibrosis transmembrane conductance regulator (CFTR).
Proteomic and ionomic profiling reveals significant alterations of protein expression and calcium homeostasis in cystic fibrosis cells.
Proteomic identification of calumenin as a G551D-CFTR associated protein.
Proteomics and Metabolomics for Cystic Fibrosis Research.
Proteomics investigation of human platelets in healthy donors and cystic fibrosis patients by shotgun nUPLC-MSE and 2DE: a comparative study.
Protocol for Application, Standardization and Validation of the Forskolin-Induced Swelling Assay in Cystic Fibrosis Human Colon Organoids.
Proton leak and CFTR in regulation of Golgi pH in respiratory epithelial cells.
PRSS1 intron mutations in patients with pancreatic cancer and chronic pancreatitis.
Pseudomonas aeruginosa alginate promotes Burkholderia cenocepacia persistence in cystic fibrosis transmembrane conductance regulator knockout mice.
Pseudomonas aeruginosa Cif Defines a Distinct Class of ?/? Epoxide Hydrolases Utilizing a His/Tyr Ring-Opening Pair.
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.
Pseudomonas aeruginosa exploits lipid A and muropeptides modification as a strategy to lower innate immunity during cystic fibrosis lung infection.
Pseudomonas aeruginosa Homoserine Lactone Activates Store-operated cAMP and Cystic Fibrosis Transmembrane Regulator-dependent Cl- Secretion by Human Airway Epithelia.
Pseudomonas aeruginosa induction of apoptosis in respiratory epithelial cells: analysis of the effects of cystic fibrosis transmembrane conductance regulator dysfunction and bacterial virulence factors.
Pseudomonas aeruginosa internalization by human epithelial respiratory cells depends on cell differentiation, polarity, and junctional complex integrity.
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.
Pseudomonas aeruginosa lipopolysaccharide induces CF-like alteration of protein secretion by human tracheal gland cells.
Pseudomonas aeruginosa pyocyanin inactivates lung epithelial vacuolar ATPase-dependent cystic fibrosis transmembrane conductance regulator expression and localization.
Pseudomonas aeruginosa sabotages the generation of host proresolving lipid mediators.
Pseudomonas aeruginosa triggers CFTR-mediated airway surface liquid secretion in swine trachea.
Pseudomonas aeruginosa-induced apoptosis is defective in respiratory epithelial cells expressing mutant cystic fibrosis transmembrane conductance regulator.
Pseudomonas Aeruginosa: Genetic Adaptation, A Strategy for its Persistence in Cystic Fibrosis.
Psoralens: novel modulators of Cl- secretion.
pSURF-2, a modified BAC vector for selective YAC cloning and functional analysis.
PTENtiating CFTR for Antimicrobial Immunity.
Pulmonary bacterial pathogens in cystic fibrosis patients and antibiotic therapy: a tool for the health workers.
Pulmonary disease caused by nontuberculous mycobacteria.
Pulmonary infections in patients with cystic fibrosis.
Pulmonary Mycobacterium avium complex infection associated with the IVS8-T5 allele of the CFTR gene.
Pulmonary nontuberculous mycobacterial disease: prospective study of a distinct preexisting syndrome.
Purification and characterization of recombinant cystic fibrosis transmembrane conductance regulator from Chinese hamster ovary and insect cells.
Purification and crystallization of the cystic fibrosis transmembrane conductance regulator (CFTR).
Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR).
Purification and reconstitution of epithelial chloride channel cystic fibrosis transmembrane conductance regulator.
Purification of CFTR for mass spectrometry analysis: identification of palmitoylation and other post-translational modifications.
Purification of the cystic fibrosis transmembrane conductance regulator protein expressed in Saccharomyces cerevisiae.
Purification, crystallization and preliminary X-ray diffraction analysis of Cif, a virulence factor secreted by Pseudomonas aeruginosa.
Purified cystic fibrosis transmembrane conductance regulator (CFTR) does not function as an ATP channel.
Purine release from spinal cord microglia after elevation of calcium by glutamate.
Purinergic receptors in airway epithelia.
Purinergic signaling underlies CFTR control of human airway epithelial cell volume.
Purinoceptor-coupled Cl- channels in mouse heart: a novel, alternative pathway for CFTR regulation.
Putative interaction of brush cells with bicarbonate secreting cells in the proximal corpus mucosa.
Pyridine nucleotide redox potential modulates cystic fibrosis transmembrane conductance regulator Cl- conductance.
Pyrophosphate stimulates wild-type and mutant cystic fibrosis transmembrane conductance regulator Cl- channels.
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.
Quantification of CFTR splice variants in adults with disseminated bronchiectasis, using the TaqMan fluorogenic detection system.
Quantification of Phenotypic Variability of Lung Disease in Children with Cystic Fibrosis.
Quantitative analysis of the packaging capacity of recombinant adeno-associated virus.
Quantitative Evaluation of CFTR Pre-mRNA Splicing Dependent on the (TG)mTn Poly-Variant Tract.
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.
Quantitative phase imaging to study transmembrane water fluxes regulated by CFTR and AQP3 in living human airway epithelial CFBE cells and CHO cells.
QUANTUM DOT SINGLE MOLECULE TRACKING REVEALS A WIDE RANGE OF DIFFUSIVE MOTIONS OF MEMBRANE TRANSPORT PROTEINS.
Quercetin increases cystic fibrosis transmembrane conductance regulator-mediated chloride transport and ciliary beat frequency: therapeutic implications for chronic rhinosinusitis.
Quorum Sensing Down-Regulation Counteracts the Negative Impact of
R-domain interactions with distal regions of CFTR lead to phosphorylation and activation.
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.
R248G cystic fibrosis transmembrane conductance regulator mutation in three siblings presenting with recurrent acute pancreatitis and reproductive issues: a case series.
Rab11b regulates the apical recycling of the cystic fibrosis transmembrane conductance regulator in polarized intestinal epithelial cells.
Rab27a negatively regulates CFTR chloride channel function in colonic epithelia: involvement of the effector proteins in the regulatory mechanism.
Rabbit erythrocytes possess adenylyl cyclase type II that is activated by the heterotrimeric G proteins Gs and Gi.
Rabbit pancreatic acini express CFTR as a cAMP-activated chloride efflux pathway.
Racially equitable diagnosis of cystic fibrosis using next-generation DNA sequencing: a case report.
RACK1 interacts with filamin-A to regulate plasma membrane levels of the cystic fibrosis transmembrane conductance regulator.
Ractopamine, a livestock feed additive, is a full agonist at trace amine-associated receptor 1.
Radioisotopic imaging allows optimization of adenovirus lung deposition for cystic fibrosis gene therapy.
Radiological analysis of children with cystic fibrosis who are homozygous for cystic fibrosis transmembrane conductance regulator mutation S549R (T-->G).
Radiotracer studies of cystic fibrosis transmembrane conductance regulator expressed in Xenopus oocytes.
Rapid and reliable genotyping of polymorphic loci modifying correct splicing of CFTR pre-mRNA using mass spectrometry.
Rapid detection of cystic fibrosis transmembrane conductance regulator gene IVS8 5T variant by real-time PCR.
Rapid endocytosis of the cystic fibrosis transmembrane conductance regulator chloride channel.
Rapid natriuretic action of aldosterone in the rat.
Rapid quantitation of gene therapy specific CFTR expression using the amplification refractory mutation system.
Rapid screening for 31 mutations and polymorphisms in the cystic fibrosis transmembrane conductance regulator gene by Lminex xMAP suspension array.
Rapid screening of genetic polymorphisms using buccal cell DNA with detection by matrix-assisted laser desorption/ionization mass spectrometry.
Rare association between cystic fibrosis, Chiari I malformation, and hydrocephalus in a baby: a case report and review of the literature.
Rat brain p64H1, expression of a new member of the p64 chloride channel protein family in endoplasmic reticulum.
Rational approach to genetic testing of cystic fibrosis (CF) in infertile men.
Rational Coupled Dynamics Network Manipulation Rescues Disease-Relevant Mutant Cystic Fibrosis Transmembrane Conductance Regulator.
Rational Design of Potent and Selective Inhibitors of an Epoxide Hydrolase Virulence Factor from Pseudomonas aeruginosa.
Rattlesnake Phospholipase A2 Increases CFTR-Chloride Channel Current and Corrects ?F508CFTR Dysfunction: Impact in Cystic Fibrosis.
Re-Imagining Cystic Fibrosis Care: Next Generation Thinking.
Re: Loss of Cystic Fibrosis Transmembrane Regulator Impairs Intestinal Oxalate Secretion.
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).
Reactive oxygen nitrogen species decrease cystic fibrosis transmembrane conductance regulator expression and cAMP-mediated Cl- secretion in airway epithelia.
Readministration of helper-dependent adenoviral vectors to mouse airway mediated via transient immunosuppression.
Real-World Long-Term Ivacaftor for Cystic Fibrosis in France: Clinical Effectiveness and Healthcare Resource Utilization.
Real-World Safety of CFTR Modulators in the Treatment of Cystic Fibrosis: A Systematic Review.
Reappraisal of Frequency of Common Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations in Iranian Cystic Fibrosis Patients.
Recent advances and new perspectives in targeting CFTR for therapy of cystic fibrosis and enterotoxin-induced secretory diarrheas.
Recent advances in cystic fibrosis.
Recent advances in developing therapeutics for cystic fibrosis.
Recent advances in the early treatment of cystic fibrosis: Bridging the gap to highly effective modulator therapy.
Recent advances in the molecular analysis of inherited disease.
Recent advances in the understanding and treatment of cystic fibrosis.
Recent advances in understanding and managing cystic fibrosis transmembrane conductance regulator dysfunction.
Recent Progress in CFTR Interactome Mapping and Its Importance for Cystic Fibrosis.
Recent Progress in the Discovery and Development of Small-Molecule Modulators of CFTR.
Recent Strategic Advances in CFTR Drug Discovery: An Overview.
Receptor activity modifying proteins regulate the activity of a calcitonin gene-related peptide receptor in rabbit aortic endothelial cells.
Receptor-mediated activation of the heterotrimeric G-protein Gs results in ATP release from erythrocytes.
Receptors that couple to 2 classes of G proteins increase cAMP and activate CFTR expressed in Xenopus oocytes.
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.
Recognition of misfolding proteins by PA700, the regulatory subcomplex of the 26 S proteasome.
Recombinant adenovirus deleted of all viral genes for gene therapy of cystic fibrosis.
Recombinant synthesis of cystic fibrosis transmembrane conductance regulator and functional nucleotide-binding domains.
Recombinant synthesis, purification, and nucleotide binding characteristics of the first nucleotide binding domain of the cystic fibrosis gene product.
Recommendations for the classification of diseases as CFTR-related disorders.
Reconstitution of a chemical defense signaling pathway in a heterologous system.
Recovery of Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction after Smoking Cessation.
Recovery of airway cystic fibrosis transmembrane conductance regulator function in mice with cystic fibrosis after single-dose lentivirus-mediated gene transfer.
Rectal epithelial expression of protein kinase A phosphorylation of cystic fibrosis transmembrane conductance regulator.
Rectification of cystic fibrosis transmembrane conductance regulator chloride channel mediated by extracellular divalent cations.
Rectification of whole cell cystic fibrosis transmembrane conductance regulator chloride current.
Recurrent Cough and Expectoration for 10 Years: A Case Report.
Red Blood Cells Under Flow Show Maximal ATP Release for Specific Hematocrit.
Redistribution of immunofluorescence of CFTR anion channel and NKCC cotransporter in chloride cells during adaptation of the killifish Fundulus heteroclitus to sea water.
Redox reagents and divalent cations alter the kinetics of cystic fibrosis transmembrane conductance regulator channel gating.
Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung.
Reduced Arylsulfatase B activity in leukocytes from cystic fibrosis patients.
Reduced blood pressure of CFTR-F508del carriers correlates with diminished arterial reactivity rather than circulating blood volume in mice.
Reduced caveolin-1 promotes hyperinflammation due to abnormal heme oxygenase-1 localization in lipopolysaccharide-challenged macrophages with dysfunctional cystic fibrosis transmembrane conductance regulator.
Reduced expression of the cystic fibrosis transmembrane conductance regulator gene in the hypothalamus of patients with Alzheimer's disease.
Reduced GM1 Ganglioside in CFTR-Deficient Human Airway Cells Results in Decreased ss1-integrin Signaling and Delayed Wound Repair.
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis.
Reduced IL-10 secretion by CD4+ T lymphocytes expressing mutant cystic fibrosis transmembrane conductance regulator (CFTR).
Reduced Intestinal Inflammation with Lumacaftor/Ivacaftor in Adolescents with Cystic Fibrosis.
Reduced microtubule acetylation in cystic fibrosis epithelial cells.
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.
Reduced NHE3-mediated Na+ absorption increases survival and decreases the incidence of intestinal obstructions in cystic fibrosis mice.
Reduced PDZ interactions of rescued ?F508CFTR increases its cell surface mobility.
Reduction of airway anion secretion via CFTR in sphingomyelin pathway.
Redundancy of mammalian proteasome beta subunit function during endoplasmic reticulum associated degradation.
Refining the continuum of CFTR-associated disorders in the era of newborn screening.
Regional Differences in Rat Conjunctival Ion Transport Activities.
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.
Regulated expression of the human CFTR gene in epithelial cells.
Regulated recycling of mutant CFTR is partially restored by pharmacological treatment.
Regulated traffic of anion transporters in mammalian Brunner's glands: a role for water and fluid transport.
Regulated trafficking of the CFTR chloride channel.
Regulating effect of TongXie-YaoFang on colonic epithelial secretion
Regulation and functional significance of airway surface liquid pH.
Regulation and properties of KCNQ1 (K(V)LQT1) and impact of the cystic fibrosis transmembrane conductance regulator.
Regulation by ATP and ADP of CFTR chloride channels that contain mutant nucleotide-binding domains.
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.
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.
Regulation of adenovirus-mediated transgene expression by the viral E4 gene products: requirement for E4 ORF3.
Regulation of amiloride-sensitive sodium absorption in murine airway epithelium by C-type natriuretic peptide.
Regulation of antiprotease and antimicrobial protein secretion by airway submucosal gland serous cells.
Regulation of apical surface fluid and protein secretion in human airway epithelial cell line Calu-3.
Regulation of Ca(2+)-activated chloride channels by cAMP and CFTR in parotid acinar cells.
Regulation of cAMP-dependent chloride channels in DC1 immortalized rabbit distal tubule cells in culture.
Regulation of CFTR Bicarbonate Channel Activity by WNK1: Implications for Pancreatitis and CFTR-Related Disorders.
Regulation of CFTR Biogenesis by the Proteostatic Network and Pharmacological Modulators.
Regulation of CFTR by protein phosphatase 2B and protein kinase C.
Regulation of CFTR channel gating.
Regulation of CFTR Chloride Channel Macroscopic Conductance by Extracellular Bicarbonate.
Regulation of CFTR Cl- channel gating by ATP binding and hydrolysis.
Regulation of CFTR Trafficking by Its R Domain.
Regulation of channel gating by AMP-activated protein kinase modulates cystic fibrosis transmembrane conductance regulator activity in lung submucosal cells.
Regulation of chemokine expression by NaCl occurs independently of cystic fibrosis transmembrane conductance regulator in macrophages.
Regulation of chloride secretion across porcine endometrial epithelial cells by prostaglandin E2.
Regulation of cholangiocyte secretion.
Regulation of Cl(-) secretion by AMPK in vivo.
Regulation of Cl- Electrolyte Permeability in Epithelia by Active Traditional Chinese Medicine Monomers for Diarrhea.
Regulation of Cl- secretion by alpha2-adrenergic receptors in mouse colonic epithelium.
Regulation of Cl-/ HCO3- exchange by cystic fibrosis transmembrane conductance regulator expressed in NIH 3T3 and HEK 293 cells.
Regulation of conductance by the number of fixed positive charges in the intracellular vestibule of the CFTR chloride channel pore.
Regulation of cortical structure by the ezrin-radixin-moesin protein family.
Regulation of cystic fibrosis transmembrane conductance regulator (CFTR) gene transcription and alternative RNA splicing in a model of developing intestinal epithelium.
Regulation of Cystic Fibrosis Transmembrane Conductance Regulator by MicroRNA-145, -223, and -494 Is Altered in ?F508 Cystic Fibrosis Airway Epithelium.
Regulation of cystic fibrosis transmembrane conductance regulator single-channel gating by bivalent PDZ-domain-mediated interaction.
Regulation of cystic fibrosis transmembrane regulator trafficking and protein expression by a Rho family small GTPase TC10.
Regulation of electrogenic anion secretion in normal and cystic fibrosis gallbladder mucosa.
Regulation of ENaC biogenesis by the stress response protein SERP1.
Regulation of endocytic trafficking and acidification are independent of the cystic fibrosis transmembrane regulator.
REGULATION OF ENDOGENOUS ENaC FUNCTIONAL EXPRESSION BY CFTR AND {Delta}F508-CFTR IN AIRWAY EPITHELIAL CELLS.
Regulation of epithelial ion channels by the cystic fibrosis transmembrane conductance regulator.
Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator.
Regulation of extracellular UTP-activated Cl- current by P2Y-PLC-PKC signaling and ATP hydrolysis in mouse ventricular myocytes.
Regulation of gap junctional communication by a pro-inflammatory cytokine in cystic fibrosis transmembrane conductance regulator-expressing but not cystic fibrosis airway cells.
Regulation of hepatic ABCC transporters by xenobiotics and in disease states.
Regulation of hepatic sulfotransferase (SULT) 1E1 expression and effects on estrogenic activity in cystic fibrosis (CF).
Regulation of human cystic fibrosis transmembrane conductance regulator (CFTR) by serum- and glucocorticoid-inducible kinase (SGK1).
Regulation of intestinal Cl- and HCO3-secretion by uroguanylin.
Regulation of intestinal gene expression by dietary zinc: induction of uroguanylin mRNA by zinc deficiency.
Regulation of intracellular pH in Calu-3 human airway cells.
Regulation of ion channels by pyridine nucleotides.
Regulation of ion transport by 5-hydroxytryptamine in rat colon.
Regulation of male fertility by CFTR and implications in male infertility.
Regulation of membrane chloride currents in rat bile duct epithelial cells.
Regulation of mRNA turnover in cystic fibrosis lung disease.
Regulation of murine cystic fibrosis transmembrane conductance regulator Cl- channels expressed in Chinese hamster ovary cells.
Regulation of plasma membrane recycling by CFTR.
Regulation of Pseudomonas aeruginosa internalization after contact lens wear in vivo and in serum-free culture by ocular surface cells.
Regulation of recombinant cardiac cystic fibrosis transmembrane conductance regulator chloride channels by protein kinase C.
Regulation of the CFTR channel by phosphorylation.
Regulation of the CFTR chloride channel from humans and sharks.
Regulation of the Coxsackie and adenovirus receptor expression is dependent on cystic fibrosis transmembrane regulator in airway epithelial cells.
Regulation of the cystic fibrosis transmembrane conductance regulator anion channel by tyrosine phosphorylation.
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.
Regulation of the cystic fibrosis transmembrane conductance regulator chloride channel by MgATP.
Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by its R domain.
Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by negative charge in the R domain.
Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by specific protein kinases and protein phosphatases.
Regulation of the depth and composition of airway surface liquid.
Regulation of the epithelial sodium channel by accessory proteins.
Regulation of the gating of cystic fibrosis transmembrane conductance regulator C1 channels by phosphorylation and ATP hydrolysis.
Regulation of the ROMK channel: interaction of the ROMK with associate proteins.
Regulation of Ubiquitin-Proteasome System mediated Degradation by Cytosolic Stress.
Regulation of vascular tone and arterial blood pressure: role of chloride transport in vascular smooth muscle.
Regulatory Crosstalk by Protein Kinases on CFTR Trafficking and Activity.
Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators.
Regulatory dynamics of 11p13 suggest a role for EHF in modifying CF lung disease severity.
Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTR.
Regulatory interaction between the cystic fibrosis transmembrane conductance regulator and HCO3- salvage mechanisms in model systems and the mouse pancreatic duct.
Regulatory interactions of N1303K-CFTR and ENaC in Xenopus oocytes: evidence that chloride transport is not necessary for inhibition of ENaC.
Regulatory mechanism underlying cyclic changes in mouse uterine bicarbonate secretion: role of estrogen.
Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions.
Relating the Disease Mutation Spectrum to the Evolution of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis.
Relation between the anatomical genital phenotype and cystic fibrosis transmembrane conductance regulator gene mutations in the absence of the vas deferens.
Relations between the frequency of the DeltaF 508 mutation and the course of pulmonary disease in cystic fibrosis patients infected with Pseudomonas aeruginosa.
Relationship between anion binding and anion permeability revealed by mutagenesis within the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator.
Relationship of a non-cystic fibrosis transmembrane conductance regulator-mediated chloride conductance to organ-level disease in Cftr(-/-) mice.
Relationships among CFTR expression, HCO3- secretion, and host defense may inform gene- and cell-based cystic fibrosis therapies.
Relationships between cystic fibrosis transmembrane conductance regulator, extracellular nucleotides and cystic fibrosis.
Relative contribution of clear cells and principal cells to luminal pH in the mouse epididymis.
Relative contribution of different transmembrane segments to the CFTR chloride channel pore.
Relative Movements of Transmembrane Regions at the Outer Mouth of the Cystic Fibrosis Transmembrane Conductance Regulator Channel Pore during Channel Gating.
Release of ATP from retinal pigment epithelial cells involves both CFTR and vesicular transport.
Relevance of genetic counselling in couples prior to intracytoplasmic sperm injection.
Relevance of variants in serum antiproteinases for the course of chronic pancreatitis.
Reliability of polymerase chain reaction (PCR) analysis of single cells for preimplantation genetic diagnosis.
Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis.
Removal of the Fe(iii) site promotes activation of the human cystic fibrosis transmembrane conductance regulator by high-affinity Zn(ii) binding.
Renal effects of glibenclamide in cystic fibrosis mice.
Renal Medullary Interstitial COX-2 (Cyclooxygenase-2) Is Essential in Preventing Salt-Sensitive Hypertension and Maintaining Renal Inner Medulla/Papilla Structural Integrity.
Repair of CFTR mRNA by spliceosome-mediated RNA trans-splicing.
Repairing mutated proteins--development of small molecules targeting defects in the cystic fibrosis transmembrane conductance regulator.
Repairing the basic defect in cystic fibrosis - one approach is not enough.
Repeat administration of an adenovirus vector encoding cystic fibrosis transmembrane conductance regulator to the nasal epithelium of patients with cystic fibrosis.
Repeat administration of DNA/liposomes to the nasal epithelium of patients with cystic fibrosis.
Repeatability and Diagnostic Value of Nasal Potential Difference in a Genetically Admixed Population.
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.
Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial.
Report of a Korean patient with cystic fibrosis, carrying Q98R and Q220X mutations in the CFTR gene.
Report of two patients with associated conditions in addition to 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.
Rescue from Pseudomonas aeruginosa Airway Infection via Stem Cell Transplantation.
Rescue of CFTR NBD2 mutants N1303K and S1235R is influenced by the functioning of the autophagosome.
Rescue of defective ATP8B1 trafficking by CFTR correctors as a therapeutic strategy for familial intrahepatic cholestasis.
Rescue of DeltaF508 and other misprocessed CFTR mutants by a novel quinazoline compound.
Rescue of DeltaF508-CFTR (cystic fibrosis transmembrane conductance regulator) by curcumin: involvement of the keratin 18 network.
Rescue of DeltaF508-CFTR by the SGK1/Nedd4-2 signaling pathway.
Rescue of F508del-CFTR by RXR motif inactivation triggers proteome modulation associated with the unfolded protein response.
Rescue of Functional CFTR Channels in Cystic Fibrosis: A Dramatic Multivalent Effect Using Iminosugar Cluster-Based Correctors.
Rescue of functional DeltaF508-CFTR channels by co-expression with truncated CFTR constructs in COS-1 cells.
Rescue of functional F508del cystic fibrosis transmembrane conductance regulator by vasoactive intestinal peptide in the human nasal epithelial cell line JME/CF15.
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.
Rescuing cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by transcomplementation.
Residual chloride secretion in intestinal tissue of deltaF508 homozygous twins and siblings with cystic fibrosis. The European CF Twin and Sibling Study Consortium.
Respiratory epithelial gene expression in patients with mild and severe cystic fibrosis lung disease.
Respiratory exacerbations in childhood associated with compound heterozygosity Phe508del/Arg117His-7T of the cystic fibrosis transmembrane regulator gene.
Respiratory syncytial virus engineered to express the cystic fibrosis transmembrane conductance regulator corrects the bioelectric phenotype of human cystic fibrosis airway epithelium in vitro.
Respiratory syncytial virus infection disrupts monolayer integrity and function in cystic fibrosis airway cells.
Respiratory syncytial virus infection in a murine model of cystic fibrosis.
Respiratory System Disease.
Response of the lungs to aspiration.
Response to Acute Lung Infection with Mucoid Pseudomonas aeruginosa in Cystic Fibrosis Mice.
Restoration of bacterial killing activity of human respiratory cystic fibrosis cells through cationic vector-mediated cystic fibrosis transmembrane conductance regulator gene transfer.
Restoration of F508-del Function by Transcomplementation: The Partners Meet in the Endoplasmic Reticulum.
Restoration of NBD1 thermal stability is necessary and sufficient to correct ?F508 CFTR folding and assembly.
Restoration of the cystic fibrosis transmembrane conductance regulator function by splicing modulation.
Restoration of W1282X CFTR activity by enhanced expression.
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.
Resveratrol ameliorates abnormalities of fluid and electrolyte secretion in a hypoxia-Induced model of acquired CFTR deficiency.
Resveratrol and ivacaftor are additive G551D CFTR-channel potentiators: therapeutic implications for cystic fibrosis sinus disease.
Resveratrol dimer trans-?-viniferin prevents rotaviral diarrhea in mice by inhibition of the intestinal calcium-activated chloride channel.
Resveratrol enhances airway surface liquid depth in sinonasal epithelium by increasing cystic fibrosis transmembrane conductance regulator open probability.
Resveratrol rescues cAMP-dependent anionic transport in the cystic fibrosis pancreatic cell line CFPAC1.
Reticulocyte lysate as a model system to study endoplasmic reticulum membrane protein degradation.
Retinal pigment epithelial function: a role for CFTR?
Retinoic acid promotes stem cell differentiation and embryonic development by transcriptionally activating CFTR.
Retinoic acid signaling regulates murine bronchial tubule formation.
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.
Reversal of cystic fibrosis phenotype in a cultured Delta508 cystic fibrosis transmembrane conductance regulator cell line by oligonucleotide insertion.
Reverse transcription-competitive multiplex PCR improves quantification of mRNA in clinical samples--application to the low abundance CFTR mRNA.
Reverse transcription-polymerase chain reaction (RT-PCR) phenotypic analysis of cell cultures of human tracheal epithelium, tracheobronchial glands, and lung carcinomas.
Reversible silencing of CFTR chloride channels by glutathionylation.
Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR.
Review of CFTR modulators 2020.
Review of Gastrointestinal Motility in Cystic Fibrosis.
Review. ATP hydrolysis-driven gating in cystic fibrosis transmembrane conductance regulator.
Revisiting CFTR inhibition: a comparative study of CFTRinh -172 and GlyH-101 inhibitors.
Revisiting cystic fibrosis transmembrane conductance regulator structure and function.
Revisiting the Role of CFTR and Counterion Permeability in the pH Regulation of Endocytic Organelles.
Rewriting CFTR to cure cystic fibrosis.
ReXSpecies--a tool for the analysis of the evolution of gene regulation across species.
Rheological effects of hypertonic saline and sodium bicarbonate solutions on cystic fibrosis sputum in vitro.
Rhesus ?-defensin-1 (RTD-1) exhibits in vitro and in vivo activity against cystic fibrosis strains of Pseudomonas aeruginosa.
Right-angle light scattering to assay basal and regulated plasma membrane Cl- conductances.
Riociguat for the treatment of Phe508del homozygous adults with cystic fibrosis.
Rising behind NO: cGMP-dependent protein kinases.
Risk of pancreatitis with mutation of the cystic fibrosis gene.
Rme-1 regulates the recycling of the cystic fibrosis transmembrane conductance regulator.
RNA Interference Screen to Identify Kinases That Suppress Rescue of ?F508-CFTR.
RNF185 is a novel E3 ligase of Endoplasmic Reticulum Associated Degradation (ERAD) that targets Cystic Fibrosis Transmembrane conductance Regulator (CFTR).
Robust method for TALEN-edited correction of pF508del in patient-specific induced pluripotent stem cells.
Robust Stimulation of W1282X-CFTR Channel Activity by a Combination of Allosteric Modulators.
Roflumilast combined with adenosine increases mucosal hydration in human airway epithelial cultures after cigarette smoke exposure.
Roflumilast partially reverses smoke-induced mucociliary dysfunction.
Roflumilast reverses CFTR-mediated ion transport dysfunction in cigarette smoke-exposed mice.
Role for cystic fibrosis transmembrane conductance regulator protein in a glutathione response to bronchopulmonary pseudomonas infection.
Role for PKC alpha and PKC epsilon in down-regulation of CFTR mRNA in a human epithelial liver cell line.
Role of actin filament organization in CFTR activation.
Role of actin in regulation of epithelial sodium channels by CFTR.
Role of ATP binding and hydrolysis in the gating of the cystic fibrosis transmembrane conductance regulator.
Role of Binding and Nucleoside Diphosphate Kinase A in the Regulation of the Cystic Fibrosis Transmembrane Conductance Regulator by AMP-Activated Protein Kinase.
Role of Ca2+ -activated ion transport in the treatment of cystic fibrosis.
Role of CD95 in pulmonary inflammation and infection in cystic fibrosis.
Role of CFTR expressed by neutrophils in modulating acute lung inflammation and injury in mice.
Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice.
Role of CFTR in autosomal recessive polycystic kidney disease.
Role of CFTR in chloride secretion across human tracheal epithelium.
Role of CFTR in oxidative stress and suicidal death of renal cells during cisplatin-induced nephrotoxicity.
Role of CFTR mutation analysis in the diagnostic algorithm for cystic fibrosis.
Role of CFTR's intrinsic adenylate kinase activity in gating of the Cl(-) channel.
Role of CFTR's PDZ1-binding domain, NBF1 and Cl(-) conductance in inhibition of epithelial Na(+) channels in Xenopus oocytes.
Role of chloride transport proteins in the vasorelaxant action of nitroprusside in isolated rat aorta.
Role of Cl channels in Cl-dependent Na/H exchange.
Role of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in patients with chronic sinopulmonary disease.
Role of cystic fibrosis transmembrane conductance regulator in pulmonary clearance of Pseudomonas aeruginosa in vivo.
Role of down-regulated in adenoma anion exchanger in HCO3- secretion across murine duodenum.
Role of epithelial sodium channels in the regulation of lung fluid homeostasis.
Role of Hsc70 binding cycle in CFTR folding and endoplasmic reticulum-associated degradation.
Role of IKK and ERK pathways in intrinsic inflammation of cystic fibrosis airways.
Role of IL-10 deficiency in excessive nuclear factor-kappaB activation and lung inflammation in cystic fibrosis transmembrane conductance regulator knockout mice.
Role of individual R domain phosphorylation sites in CFTR regulation by protein kinase A.
Role of inflammation and oxidative stress in tissue damage associated with cystic fibrosis: CAPE as a future therapeutic strategy.
Role of Interaction and Nucleoside Diphosphate Kinase B in Regulation of the Cystic Fibrosis Transmembrane Conductance Regulator Function by cAMP-Dependent Protein Kinase A.
Role of membrane trafficking in plasma membrane solute transport.
Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections.
Role of natriuretic peptides in ion transport mechanisms.
Role of NHERF1, cystic fibrosis transmembrane conductance regulator, and cAMP in the regulation of aquaporin 9.
Role of oxygen availability in CFTR expression and function.
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.
Role of phosphodiesterase-4 inhibitors in chronic obstructive pulmonary disease.
Role of pirfenidone in TGF-? pathways and other inflammatory pathways in acute respiratory syndrome coronavirus 2 (SARS-Cov-2) infection: a theoretical perspective.
Role of Protein Kinase A-Mediated Phosphorylation in CFTR Channel Activity Regulation.
Role of protein phosphatases in the activation of CFTR (ABCC7) by genistein and bromotetramisole.
Role of purinergic receptors in chloride secretion in Caco-2 cells.
Role of Quercetin in Modulating Chloride Transport in the Intestine.
Role of Smad3 and p38 Signalling in Cigarette Smoke-induced CFTR and BK dysfunction in Primary Human Bronchial Airway Epithelial Cells.
Role of sphingosine-1-phosphate phosphohydrolase 1 in the regulation of resistance artery tone.
Role of the actin cytoskeleton in the regulation of the cystic fibrosis transmembrane conductance regulator.
Role of the amiloride-sensitive epithelial Na+ channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease.
Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections.
Role of the cystic fibrosis transmembrane conductance regulator in internalization of Pseudomonas aeruginosa by polarized respiratory epithelial cells.
Role of the energy sensor AMP-activated protein kinase in renal physiology and disease.
Role of the extracellular loop in the folding of a CFTR transmembrane helical hairpin.
Role of the juxtamembrane region of cytoplasmic loop 3 in the gating and conductance of the cystic fibrosis transmembrane conductance regulator chloride channel.
Role of the PDZ scaffolding protein in tubule cells in maintenance of polarised function.
Role of the scaffold protein RACK1 in apical expression of CFTR.
Role of the ubiquitin system in regulating ion transport.
Role of tyrosine phosphorylation in the muscarinic activation of the cystic fibrosis transmembrane conductance regulator (CFTR).
Roles of CLCA and CFTR in electrolyte re-absorption from rat saliva.
Roles of volume-regulatory anion channels, VSOR and Maxi-Cl, in apoptosis, cisplatin resistance, necrosis, ischemic cell death, stroke and myocardial infarction.
RPTOR, a novel target of miR-155, elicits a fibrotic phenotype of cystic fibrosis lung epithelium by upregulating CTGF.
Rutaecarpine induces chloride secretion across rat isolated distal colon.
S-carbocysteine-lysine salt monohydrate and cAMP cause non-additive activation of the cystic fibrosis transmembrane regulator channel in human respiratory epithelium.
S-CMC-Lys-dependent stimulation of electrogenic glutathione secretion by human respiratory epithelium.
S-nitrosoglutathione increases cystic fibrosis transmembrane regulator maturation.
S-Nitrosothiols increases cystic fibrosis transmembrane regulator expression and maturation in the cell surface.
S-nitrosylating agents: a novel class of compounds that increase cystic fibrosis transmembrane conductance regulator expression and maturation in epithelial cells.
S737F is a new CFTR mutation typical of patients originally from the Tuscany region in Italy.
Saccharomyces cerivisiae as a model system for kidney disease: what can yeast tell us about renal function?
Safety and biological efficacy of an adeno-associated virus vector-cystic fibrosis transmembrane regulator (AAV-CFTR) in the cystic fibrosis maxillary sinus.
Safety and efficacy of the cystic fibrosis transmembrane conductance regulator potentiator icenticaftor (QBW251).
Safety and efficacy of treatment with lumacaftor in combination with ivacaftor in younger patients with cystic fibrosis.
Safety of adenovirus-mediated transfer of the human cystic fibrosis transmembrane conductance regulator cDNA to the lungs of nonhuman primates.
Safety of aerosolized INS 365 in patients with mild to moderate cystic fibrosis: results of a phase I multi-center study.
Safety of airway gene transfer with Ad2/CFTR2: aerosol administration in the nonhuman primate.
Safety of local delivery of low- and intermediate-dose adenovirus gene transfer vectors to individuals with a spectrum of morbid conditions.
Safety of single-dose administration of an adeno-associated virus (AAV)-CFTR vector in the primate lung.
Salinity-dependent expression of ncc2 in opercular epithelium and gill of mummichog (Fundulus heteroclitus).
Salivary Biomarkers and Oral Microbial Load in Relation to the Dental Status of Adults with Cystic Fibrosis.
Salivary electrolyte concentrations are associated with cystic fibrosis transmembrane regulator genotypes.
Salivary Thiocyanate as a Biomarker of Cystic Fibrosis Transmembrane Regulator Function.
Salmeterol Restores Secretory Functions in Cystic Fibrosis Airway Submucosal Gland Serous Cells.
Salmonella enterica serovar typhi modulates cell surface expression of its receptor, the cystic fibrosis transmembrane conductance regulator, on the intestinal epithelium.
Salmonella typhi uses CFTR to enter intestinal epithelial cells.
Salt stress increases abundance and glycosylation of CFTR localized at apical surfaces of salt gland secretory cells.
Same Game, Different Players: Emerging Pathogens of the CF Lung.
SARS-CoV-2 may hijack GPCR signaling pathways to dysregulate lung ion and fluid transport.
Scaffolding protein GOPC regulates tight junction structure.
Scanning the cystic fibrosis transmembrane conductance regulator gene using high-resolution DNA melting analysis.
Screen-Printed Sensor for Low-Cost Chloride Analysis in Sweat for Rapid Diagnosis and Monitoring of 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.
Screening for cystic fibrosis transmembrane conductance regulator gene mutations in men included in an intracytoplasmic sperm injection programme.
Screening for cystic fibrosis: the importance of using the correct tools.
Screening for F508del as a first step in the molecular diagnosis of cystic fibrosis.
Screening for mutations in the cystic fibrosis transmembrane regulator gene in an infertility clinic.
Screening for non-delta F508 mutations in five exons of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in Italy.
Screening for regulatory variants in 460kb encompassing the CFTR locus in cystic fibrosis patients.
Screening methods for cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in non-human primates.
Screening of ?F508 mutation and IVS8-poly T polymorphism in CFTR gene in Tunisian infertile men without CBAVD.
Screening of Two Neighboring
Screening practices for mutations in the CFTR gene ABCC7.
Search for mutations in pancreatic sufficient cystic fibrosis Italian patients: detection of 90% of molecular defects and identification of three novel mutations.
Searching for a cure for cystic fibrosis. A 25-year quest in a nutshell.
Sec16 in Conventional and Unconventional Exocytosis: Working at the Interface of Membrane Traffic and Secretory Autophagy?
Secretion of ATP from Schwann cells in response to uridine triphosphate.
Secretory cells of the airway express molecules of the chemoreceptive cascade.
Secretory diarrhoea: mechanisms and emerging therapies.
Secretory low-molecular-weight phospholipases A2 and their specific receptor in bile ducts of patients with intrahepatic calculi: factors of chronic proliferative cholangitis.
Segmental heterogeneity of electrogenic secretions in human ascending colon and rectum.
Segregation of delta F508 and normal CFTR alleles in human sperm.
Selective activation of cystic fibrosis transmembrane conductance regulator Cl- and HCO3- conductances.
Selective Binding of HSC70 and its Co-Chaperones to Structural Hotspots on CFTR.
Selective block of swelling-activated Cl- channels over cAMP-dependent Cl- channels in ventricular myocytes.
Selectively targeting key inflammatory pathways in cystic fibrosis.
Self-assembled peptide-poloxamine nanoparticles enable in vitro and in vivo genome restoration for cystic fibrosis.
Self-Reactive CFTR T Cells in Humans: Implications for Gene Therapy.
Self-renewal and differentiation of rat Epididymal basal cells using a novel in vitro organoid model.
Seminal plasma characteristics as indicators of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in men with obstructive azoospermia.
Seminal vesicle agenesis: An uncommon cause of azoospermia.
Sendai virus-mediated CFTR gene transfer to the airway epithelium.
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.
Sequence analyses of presenilin mutations linked to familial Alzheimer's disease.
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.
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.
Sequence and structural homology among membrane-associated domains of CFTR and certain transporter proteins.
Sequence homologies between nucleotide binding regions of CFTR and G-proteins suggest structural and functional similarities.
Sequence Hydropathy Dominates Membrane Protein Response to Detergent Solubilization.
Sequence specific generation of a DNA panhandle permits PCR amplification of unknown flanking DNA.
Sequence-specific retention and regulated integration of a nascent membrane protein by the endoplasmic reticulum Sec61 translocon.
Sequences within the coding regions of clotting factor VIII and CFTR block transcriptional elongation.
Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator.
SERCA Pump Inhibitors Do Not Correct Biosynthetic Arrest of {Delta}F508 CFTR in Cystic Fibrosis.
Sertoli cell expression of the cystic fibrosis transmembrane conductance regulator.
Serum and glucocorticoid-inducible kinase1 increases plasma membrane wt-CFTR in human airway epithelial cells by inhibiting its endocytic retrieval.
Serum inflammatory profiles in cystic fibrosis mice with and without Bordetella pseudohinzii infection.
Serum Proteomic Analysis from Bacteremic and Leucopenic Rabbits.
Serum- and glucocorticoid-induced protein kinase 1 (SGK1) increases the cystic fibrosis transmembrane conductance regulator (CFTR) in airway epithelial cells by phosphorylating Shank2E protein.
Severe allergic bronchopulmonary aspergillosis in an infant with cystic fibrosis and her asthmatic father.
Severe cystic fibrosis associated with a deltaF508/R347H + D979A compound heterozygous genotype.
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.
Severe osteopenia in CFTR-null mice.
Severed channels probe regulation of gating of cystic fibrosis transmembrane conductance regulator by its cytoplasmic domains.
Severed molecules functionally define the boundaries of the cystic fibrosis transmembrane conductance regulator's NH(2)-terminal nucleotide binding domain.
Sex hormones regulate CFTR in developing fetal rat lung epithelial cells.
Sexual and reproductive health in cystic fibrosis.
Sexual maturation and changes in water and salt transport components in the kidney and intestine of three-spined stickleback (Gasterosteus aculeatus L.).
SFPQ rescues F508del-CFTR expression and function in cystic fibrosis bronchial epithelial cells.
Shank2 Mutant Mice Display a Hypersecretory Response to Cholera Toxin.
Short communication: novel truncating mutations in the CFTR gene causing a severe form of cystic fibrosis in Italian patients.
Short- and long-term low-salinity acclimation effects on the branchial and intestinal gene expression in the European seabass (Dicentrarchus labrax).
Short-chain fatty acids affect cystic fibrosis airway inflammation and bacterial growth.
Short-term consequences of F508del-CFTR thermal instability on CFTR-dependent transepithelial currents in human airway epithelial cells.
Short-term low-salinity tolerance by the longhorn sculpin, Myoxocephalus octodecimspinosus.
Shrinkage activates a nonselective conductance: involvement of a Walker-motif protein and PKC.
Side chain and backbone contributions of Phe508 to CFTR folding.
Signaling Cascade Involved in Rapid Stimulation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Dexamethasone.
Signature current of SO2-induced bronchitis in rabbit.
Signature motifs identify an acinetobacter cif virulence factor with epoxide hydrolase activity.
Silencing of the Hsp70-specific nucleotide-exchange factor BAG3 corrects the F508del-CFTR variant by restoring autophagy.
Simple binding of protein kinase A prior to phosphorylation allows CFTR anion channels to be opened by nucleotides.
Simple method for haplotyping the poly(TG) repeat in individuals carrying the IVS8 5T allele in the CFTR gene.
Simple multiplex genotyping by surface-enhanced resonance Raman scattering.
Simulation analysis of intracellular Na+ and Cl- homeostasis during beta 1-adrenergic stimulation of cardiac myocyte.
Simulation of Cl(-) Secretion in Epithelial Tissues: New Methodology Estimating Activity of Electro-Neutral Cl(-) Transporter.
Simultaneous cycle sequencing assessment of (TG)m and Tn tract length in CFTR gene.
Simultaneous screening for 11 mutations in the cystic fibrosis transmembrane conductance regulator gene by multiplex amplification and reverse dot-blot.
Single intragenic microsatellite preimplantation genetic diagnosis for cystic fibrosis provides positive allele identification of all CFTR genotypes for informative couples.
Single-cell-resolved differentiation of human induced pluripotent stem cells into pancreatic duct-like organoids on a microwell chip.
Single-dose lentiviral gene transfer for lifetime airway gene expression.
Single-Gene Sequencing in Newborn Screening: Success, Challenge, Hope.
Single-particle tracking of membrane protein diffusion in a potential: simulation, detection, and application to confined diffusion of CFTR Cl- channels.
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.
Single-stranded conformation polymorphism analysis of the CFTR gene in Slovenian cystic fibrosis patients: detection of mutations and sequence variations.
Sinus hypoplasia in the cystic fibrosis rat resolves in the absence of chronic infection.
Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis.
Sites associated with Kalydeco binding on human Cystic Fibrosis Transmembrane Conductance Regulator revealed by Hydrogen/Deuterium Exchange.
Sixty-five years since the New York heat wave: advances in sweat testing for cystic fibrosis.
Skeletal muscle contractility and fatigability in adults with cystic fibrosis.
Skin ionocyte remodeling in the amphibious mangrove rivulus fish (Kryptolebias marmoratus).
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.
Skipping of multiple CFTR exons is not a result of single exon omissions.
SLC26 anion exchangers in uterine epithelial cells and spermatozoa: clues from the past and hints to the future.
SLC26 anion exchangers of guinea pig pancreatic duct: molecular cloning and functional characterization.
SLC26A Gene Family Participate in pH Regulation during Enamel Maturation.
Slc26a11 is prominently expressed in the brain and functions as a chloride channel: expression in Purkinje cells and stimulation of V H(+)-ATPase.
Slc26a3 deficiency is associated with epididymis dysplasia and impaired sperm fertilization potential in the mouse.
Slc26a9 is inhibited by the R-region of the cystic fibrosis transmembrane conductance regulator via the STAS domain.
SLC9A3 Affects Vas Deferens Development and Associates with Taiwanese Congenital Bilateral Absence of the Vas Deferens.
Sleep-Related Rhythmic Movement Disorder in Triplets: Evidence for Genetic Predisposition?
Slow conversions among subconductance states of cystic fibrosis transmembrane conductance regulator chloride channel.
Small changes in gene expression of targeted osmoregulatory genes when exposing marine and freshwater threespine stickleback (Gasterosteus aculeatus) to abrupt salinity transfers.
Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway.
Small heat-shock proteins select deltaF508-CFTR for endoplasmic reticulum-associated degradation.
Small Hsps as Therapeutic Targets of Cystic Fibrosis Transmembrane Conductance Regulator Protein.
Small interfering peptide (siP) for in vivo examination of the developing lung interactonome.
Small linear chloride channels are endogenous to nonepithelial cells.
Small Molecule Anion Carriers Correct Abnormal Airway Surface Liquid Properties in Cystic Fibrosis Airway Epithelia.
Small-molecule CFTR activators increase tear secretion and prevent experimental dry eye disease.
Small-molecule CFTR inhibitors slow cyst growth in polycystic kidney disease.
Small-molecule inhibitors of the cystic fibrosis transmembrane conductance regulator increase pancreatic endocrine cell development in rat and mouse.
Small-molecule ion channels increase host defences in cystic fibrosis airway epithelia.
Small-molecule screen identifies inhibitors of a human intestinal calcium-activated chloride channel.
Small-Molecule Screening for Genetic Diseases.
Small-molecule vasopressin-2 receptor antagonist identified by a g-protein coupled receptor "pathway" screen.
Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation.
Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR.
Solute transporters and aquaporins are impaired in celiac disease.
Some like it hot: curcumin and CFTR.
Spatiotemporal coupling of cAMP transporter to CFTR chloride channel function in the gut epithelia.
Specialized Pro-Resolving Lipid Mediators in Cystic Fibrosis.
Specialized Pro-resolving Mediators Regulate Alveolar Fluid Clearance during Acute Respiratory Distress Syndrome.
Species-specific differences in mouse and human airway epithelial biology of recombinant adeno-associated virus transduction.
Specific rescue of cystic fibrosis transmembrane conductance regulator processing mutants using pharmacological chaperones.
Specific resistance to Pseudomonas aeruginosa infection in zebrafish is mediated by the cystic fibrosis transmembrane conductance regulator.
Specific stabilization of CFTR by phosphatidylserine.
Specificity of NHERF1 regulation of GPCR signaling and function in human airway smooth muscle.
Spectrum and distribution of CFTR gene mutations in asthma and chronic pancreatitis cases of North Indian population.
Spectrum of CFTR gene sequence variants in a northern Portugal population.
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.
Spectrum of CFTR mutations in Mexican cystic fibrosis patients: identification of five novel mutations (W1098C, 846delT, P750L, 4160insGGGG and 297-1G-->A).
Spectrum of cystic fibrosis mutations in Serbia and Montenegro and strategy for prenatal diagnosis.
Spectrum of cystic fibrosis mutations in syrian patients.
Spectrum of ion channels in alveolar epithelial cells: implications for alveolar fluid balance.
Spectrum of mutations and variants/haplotypes of CFTR and genotype-phenotype correlation in idiopathic chronic pancreatitis and controls in Chinese by complete analysis.
Speeding Up the Identification of Cystic Fibrosis Transmembrane Conductance Regulator-Targeted Drugs: An Approach Based on Bioinformatics Strategies and Surface Plasmon Resonance.
Sperm cystic fibrosis transmembrane conductance regulator expression level is relevant to fecundity of healthy couples.
Sperm retrieval and intracytoplasmic sperm injection outcomes in men with cystic fibrosis disease versus congenital bilateral absence of the vas deferens.
Sphingolipids and plasma membrane hydrolases in human primary bronchial cells during differentiation and their altered patterns in cystic fibrosis.
Sphingomyelinase decreases transepithelial anion secretion in airway epithelial cells in part by inhibiting CFTR-mediated apical conductance.
Sphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity.
Sphingosine-1-Phosphate Signaling Regulates Myogenic Responsiveness in Human Resistance Arteries.
SPI-0211 activates T84 cell chloride transport and recombinant human ClC-2 chloride currents.
Spiperone, identified through compound screening, activates calcium-dependent chloride secretion in the airway.
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.
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.
Splicing defects in the CFTR gene: Minigene analysis of two mutations, 1811+1G>C and 1898+3A>G.
Splicing factors induce cystic fibrosis transmembrane regulator exon 9 skipping through a nonevolutionary conserved intronic element.
SPLUNC1 degradation by the cystic fibrosis mucosal environment drives airway surface liquid dehydration.
Spontaneous hypoglycemia in patients with cystic fibrosis.
SPX-101 Is a Novel Epithelial Sodium Channel-targeted Therapeutic for Cystic Fibrosis That Restores Mucus Transport.
Src signaling links mediators of inflammation to Cx43 gap junction channels in primary and transformed CFTR-expressing airway cells.
STa and cGMP stimulate CFTR translocation to the surface of villus enterocytes in rat jejunum and is regulated by protein kinase G.
Stability Prediction for Mutations in the Cytosolic Domains of Cystic Fibrosis Transmembrane Conductance Regulator.
Stabilization of a nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator yields insight into disease-causing mutations.
Stable ATP binding mediated by a partial NBD dimer of the CFTR chloride channel.
Stable in vivo expression of the cystic fibrosis transmembrane conductance regulator with an adeno-associated virus vector.
Standardized Measurement of Nasal Membrane Transepithelial Potential Difference (NPD).
State-dependent access of anions to the cystic fibrosis transmembrane conductance regulator chloride channel pore.
State-dependent blocker interactions with the CFTR chloride channel: implications for gating the pore.
State-dependent chemical reactivity of an engineered cysteine reveals conformational changes in the outer vestibule of the cystic fibrosis transmembrane conductance regulator.
State-dependent inhibition of cystic fibrosis transmembrane conductance regulator chloride channels by a novel peptide toxin.
State-dependent modulation of CFTR gating by pyrophosphate.
Status of fluid and electrolyte absorption in cystic fibrosis.
Stem cell-derived organoids to model gastrointestinal facets of cystic fibrosis.
Steviol Reduces MDCK Cyst Formation and Growth by Inhibiting CFTR Channel Activity and Promoting Proteasome-Mediated CFTR Degradation.
Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease.
Stimulation by alkylxanthines of chloride efflux in CFPAC-1 cells does not involve A1 adenosine receptors.
Stimulation by caveolin-1 of the hypotonicity-induced release of taurine and ATP at basolateral, but not apical, membrane of Caco-2 cells.
Stimulation effect of wide type CFTR chloride channel by the naturally occurring flavonoid tangeretin.
Stimulation of alveolar epithelial fluid clearance in human lungs by exogenous epinephrine.
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.
Stimulation of CFTR activity by its phosphorylated R domain.
Stimulation of chloride secretion by baicalein in isolated rat distal colon.
Stimulation of Cl(-) secretion by chlorzoxazone.
Stimulation of cystic fibrosis transmembrane conductance regulator-dependent short-circuit currents across DeltaF508 murine intestines.
Stimulation of epithelial sodium channel activity by the sulfonylurea glibenclamide.
Stimulation of Intestinal Cl- Secretion Through CFTR by Caffeine Intake in Salt-Sensitive Hypertensive Rats.
Stimulation of nuclear receptor peroxisome proliferator-activated receptor-? limits NF-?B-dependent inflammation in mouse cystic fibrosis biliary epithelium.
Stimulation of rat erythrocyte P2X7 receptor induces the release of epoxyeicosatrienoic acids.
Stimulation of the cystic fibrosis transmembrane regulator expression by estrogen in vivo.
Stimulation of Wild-Type, F508del- and G551D-CFTR Chloride Channels by Non-Toxic Modified pyrrolo[2,3-b]pyrazine Derivatives.
Stimulation of Xenopus P2Y1 receptor activates CFTR in A6 cells.
Stimulatory and inhibitory protein kinase C consensus sequences regulate the cystic fibrosis transmembrane conductance regulator.
Stoichiometry and novel gating mechanism within the cystic fibrosis transmembrane conductance regulator channel.
Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro.
Strain-dependent pulmonary gene expression profiles of a cystic fibrosis mouse model.
Strain-specific variants of the mouse Cftr promoter region reveal transcriptional regulatory elements.
Strategies against Nonsense: Oxadiazoles as Translational Readthrough-Inducing Drugs (TRIDs).
Strategies for cystic fibrosis transmembrane conductance regulator inhibition: from molecular mechanisms to treatment for secretory diarrhoeas.
Strategies for the etiological therapy of cystic fibrosis.
Strategies for Unambiguous Detection of Allelic Heterozygosity via Direct DNA Sequencing of PCR Products: Application to the HLA DRB1 Locus.
Strategies to inhibit cyst formation in ADPKD.
Strategies to investigate the mechanism of action of CFTR modulators.
Stratifying infants with cystic fibrosis for disease severity using intestinal organoid swelling as a biomarker of CFTR function.
Streptomycin treatment alters the intestinal microbiome, pulmonary T cell profile and airway hyperresponsiveness in a cystic fibrosis mouse model.
Strong toll-like receptor responses in cystic fibrosis patients are associated with higher lung function.
Structural analog of sildenafil identified as a novel corrector of the F508del-CFTR trafficking defect.
Structural analogues of roscovitine rescue the intracellular traffic and the function of ER-retained ABCB4 variants in cell models.
Structural analysis of cloned plasma membrane proteins by freeze-fracture electron microscopy.
Structural and ionic determinants of 5-nitro-2-(3-phenylprophyl-amino)-benzoic acid block of the CFTR chloride channel.
Structural basis for adenylate kinase activity in ABC ATPases.
Structural basis for misfolding at a disease phenotypic position in CFTR: Comparison of TM3/4 helix-loop-helix constructs with TM4 peptides.
Structural basis for specificity and potency of xanthine derivatives as activators of the CFTR chloride channel.
Structural basis for the channel function of a degraded ABC transporter, CFTR (ABCC7).
Structural basis of the Na+/H+ exchanger regulatory factor PDZ1 interaction with the carboxyl-terminal region of the cystic fibrosis transmembrane conductance regulator.
Structural basis of typhoid: Salmonella typhi type IVb pilin (PilS) and cystic fibrosis transmembrane conductance regulator interaction.
Structural biology of Rad50 ATPase: ATP-driven conformational control in DNA double-strand break repair and the ABC-ATPase superfamily.
Structural Changes Fundamental to Gating of the Cystic Fibrosis Transmembrane Conductance Regulator Anion Channel Pore.
Structural changes of CFTR R region upon phosphorylation: a plastic platform for intramolecular and intermolecular interactions.
Structural cues involved in endoplasmic reticulum degradation of G85E and G91R mutant cystic fibrosis transmembrane conductance regulator.
Structural determinants for activation and block of CFTR-mediated chloride currents by apigenin.
Structural effects of extracellular loop mutations in CFTR helical hairpins.
Structural features of Escherichia coli heat-stable enterotoxin that activates membrane-associated guanylyl cyclase.
Structural identification of a hotspot on CFTR for potentiation.
Structural mechanisms for defective CFTR gating caused by the Q1412X mutation, a severe Class VI pathogenic mutation in cystic fibrosis.
Structural models of CFTR-AMPK and CFTR-PKA interactions: R-domain flexibility is a key factor in CFTR regulation.
Structural stability of purified human CFTR is systematically improved by mutations in nucleotide binding domain 1.
Structure and dynamics of NBD1 from CFTR characterized using crystallography and hydrogen/deuterium exchange mass spectrometry.
Structure and expression of the messenger RNA encoding the murine multidrug resistance protein, an ATP-binding cassette transporter.
Structure and function of the CFTR chloride channel.
Structure and function of the cystic fibrosis transmembrane conductance regulator.
Structure and in vitro substrate specificity of the murine multidrug resistance-associated protein.
Structure and regulation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in killifish: A comparative genomics approach.
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator.
Structure of the human MDR3 gene and physical mapping of the human MDR locus.
Structure of Transmembrane Helix 8 and Possible Membrane Defects in CFTR.
STRUCTURE, GATING, AND REGULATION OF THE CFTR ANION CHANNEL.
Structure-activity relationship of 1,4-dihydropyridines as potentiators of the cystic fibrosis transmembrane conductance regulator chloride channel.
Structure-activity relationships of cyanoquinolines with corrector-potentiator activity in ?F508 cystic fibrosis transmembrane conductance regulator protein.
Structure-function analysis of a double-mutant cystic fibrosis transmembrane conductance regulator protein occurring in disorders related to cystic fibrosis.
Structure-guided combination therapy to potently improve the function of mutant CFTRs.
Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant.
Studies of the role for NSP4 in the pathogenesis of homologous murine rotavirus diarrhea.
Studies on human porin. IX. Immunolocalization of porin and CFTR channels in human surface respiratory epithelium.
Study of mutant and polyvariant mutant CFTR genes in patients with congenital absence of the vas deferens.
Subcellular distribution of CFTR in rat intestine supports a physiologic role for CFTR regulation by vesicle traffic.
Subcellular localization of CFTR to endosomes in a ductal epithelium.
Subepithelial fibrosis and degradation of the bronchial extracellular matrix in 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.
Submucosal gland distribution in the mouse has a genetic determination localized on chromosome 9.
Submucosal glands and airway defense.
Substance P stimulates CFTR-dependent fluid secretion by mouse tracheal submucosal glands.
Substitution of Yor1p NBD1 residues improves the thermal stability of Human Cystic Fibrosis Transmembrane Conductance Regulator.
Substrates and inhibitors of human multidrug resistance associated proteins and the implications in drug development.
Substrates of multidrug resistance-associated proteins block the cystic fibrosis transmembrane conductance regulator chloride channel.
Substratum stiffness tunes membrane voltage in mammary epithelial cells.
Successful targeting of the mouse cystic fibrosis transmembrane conductance regulator gene in embryonal stem cells.
Suitability of oligonucleotide-mediated cystic fibrosis gene repair in airway epithelial cells.
Sulfation of chondroitin/dermatan sulfate by cystic fibrosis pancreatic duct cells is not different from control cells.
Sulfonylurea receptors inhibit the epithelial sodium channel (ENaC) by reducing surface expression.
Sulfonylurea-sensitive K(+) transport is involved in Cl(-) secretion and cyst trowth by cultured ADPKD cells.
SUMOylation represses the transcriptional activity of the Unfolded Protein Response transducer ATF6.
Suppression of adenosine-activated chloride transport by ethanol in airway epithelia.
Suppression of CFTR-mediated Cl Secretion of Airway Epithelium in Vitamin C-deficient Mice.
Suppression of CFTR-mediated Cl(-) secretion by enhanced expression of epithelial Na(+) channels in mouse endometrial epithelium.
Suppressive effects of 4-phenylbutyrate on the aggregation of Pael receptors and endoplasmic reticulum stress.
Suppressor Mutations in the Transmembrane Segments of P-glycoprotein Promote Maturation of Processing Mutants and Disrupt a Subset of Drug-binding Sites.
Supramolecular Transmembrane Anion Transport: New Assays and Insights.
Surface expression of the cystic fibrosis transmembrane conductance regulator mutant DeltaF508 is markedly upregulated by combination treatment with sodium butyrate and low temperature.
Survey of cystic fibrosis transmembrane conductance regulator genotypes in primary sclerosing cholangitis.
Survey of patients with cystic fibrosis and caregivers decisions regarding CFTR modulators.
Susceptibility to nontuberculous mycobacterial lung disease.
Susceptibility to typhoid fever is associated with a polymorphism in the cystic fibrosis transmembrane conductance regulator (CFTR).
Sustained Glycemic Control With Ivacaftor in Cystic Fibrosis-Related Diabetes.
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.
Sweat chloride and immunoreactive trypsinogen in infants carrying two CFTR mutations and not affected by cystic fibrosis.
Sweat Chloride Testing and Nasal Potential Difference (NPD) Are Primary Outcome Parameters in Treatment with Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators.
Swelling-activated Ca2+ entry via TRPV4 channel is defective in cystic fibrosis airway epithelia.
Swelling-activated, cystic fibrosis transmembrane conductance regulator-augmented ATP release and Cl- conductances in murine C127 cells.
Swelling-induced Cl- current in guinea-pig atrial myocytes: inhibition by glibenclamide.
Swelling-induced, CFTR-independent ATP release from a human epithelial cell line: lack of correlation with volume-sensitive cl(-) channels.
Symmetric Snapback Primers for Scanning and Genotyping of the Cystic Fibrosis Transmembrane Conductance Regulator Gene.
Synaptic scaffolding molecule binds to and regulates vasoactive intestinal polypeptide type-1 receptor in epithelial cells.
Synergic action of insulin and genistein on Na+/K+/2Cl- cotransporter in renal epithelium.
Synergistic activation of guinea-pig cardiac cystic fibrosis transmembrane conductance regulator by the tyrosine kinase inhibitor genistein and cAMP.
Synergistic effects of cAMP- and calcium-mediated amylase secretion in isolated pancreatic acini from cystic fibrosis mice.
Synergistic effects of cystic fibrosis transmembrane conductance regulator and aquaporin-9 in the rat epididymis.
Synergistic effects of interferon gamma and tumour necrosis factor alpha on T84 cell function.
Synergistic post-transcriptional regulation of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) by miR-101 and miR-494 specific binding.
Synergistic Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Gating by Two Chemically Distinct Potentiators, Ivacaftor (VX-770) and 5-Nitro-2-(3-Phenylpropylamino) Benzoate.
Synergy of cAMP and calcium signaling pathways in CFTR regulation.
Synergy-based small-molecule screen using a human lung epithelial cell line yields ?F508-CFTR correctors that augment VX-809 maximal efficacy.
Synonymous Codon Usage Affects the Expression of Wild Type and F508del CFTR.
Synonymous mutations in CFTR exon 12 affect splicing and are not neutral in evolution.
Syntaxin 16 binds to CFTR and regulates its membrane trafficking in epithelial cells.
Syntaxin 1A inhibits regulated CFTR trafficking in xenopus oocytes.
Syntaxin 3 is necessary for cAMP and cGMP-regulated exocytosis of CFTR: implications for enterotoxigenic diarrhea.
Syntaxin 6 and CAL mediate the degradation of the cystic fibrosis transmembrane conductance regulator.
Syntaxin 8 impairs trafficking of cystic fibrosis transmembrane conductance regulator (CFTR) and inhibits its channel activity.
Synthesis and biological evaluation of thiazole derivatives on basic defects underlying cystic fibrosis.
Synthesis and evaluation of esterified Hsp70 agonists in cellular models of protein aggregation and folding.
Synthesis and properties of molecular probes for the rescue site on mutant cystic fibrosis transmembrane conductance regulator.
Synthesis and structure-activity relationship of aminoarylthiazole derivatives as potential potentiators of the chloride transport defect in cystic fibrosis.
Synthesis of pentasaccharides corresponding to the glycoform II of the outer core region of the Pseudomonas aeruginosa lipopolysaccharide.
Synthesis, SAR, crystal structure, and biological evaluation of benzoquinoliziniums as activators of wild-type and mutant cystic fibrosis transmembrane conductance regulator channels.
Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor.
Systemic inflammatory mediators and cystic fibrosis genotype.
Systemic versus tissue-level prolactin signaling in a teleost during a tidal cycle.
SYVN1, NEDD8, and FBXO2 Proteins Regulate ?F508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Ubiquitin-mediated Proteasomal Degradation.
TALEN-Mediated Gene Targeting for Cystic Fibrosis-Gene Therapy.
Tandem mass tag-based quantitative proteomic analysis of the liver reveals potential protein targets of Xiaochaihutang in CUMS model of depression.
Tanshinone IIA sulfonate protects against cigarette smoke-induced COPD and down-regulation of CFTR in mice.
Targeted Activation of Cystic Fibrosis Transmembrane Conductance Regulator.
Targeted correction of a defective selectable marker gene in human epithelial cells by small DNA fragments.
Targeted degradation of ABC transporters in health and disease.
Targeted extended cystic fibrosis mutation testing on known and at-risk patients and relatives.
Targeted Gene Next-Generation Sequencing in Chinese Children with Chronic Pancreatitis and Acute Recurrent Pancreatitis.
Targeted inactivation of cystic fibrosis transmembrane conductance regulator chloride channel gene prevents ischemic preconditioning in isolated mouse heart.
Targeted Next-Generation Sequencing Effectively Analyzed the Cystic Fibrosis Transmembrane Conductance Regulator Gene in Pancreatitis.
Targeted proteomic quantitation of the absolute expression and turnover of cystic fibrosis transmembrane conductance regulator in the apical plasma membrane.
Targeted quantitation of overexpressed and endogenous cystic fibrosis transmembrane conductance regulator using multiple reaction monitoring tandem mass spectrometry and oxygen stable isotope dilution.
Targeted therapies to improve CFTR function in cystic fibrosis.
Targeted therapy for chronic respiratory disease: a new paradigm.
Targeted therapy for cystic fibrosis: cystic fibrosis transmembrane conductance regulator mutation-specific pharmacologic strategies.
Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis.
Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ?F508 cystic fibrosis transmembrane conductance regulator.
Targeting CAL as a negative regulator of DeltaF508-CFTR cell-surface expression: an RNA interference and structure-based mutagenetic approach.
Targeting cell-specific gene expression with an adenovirus vector containing the lacZ gene under the control of the CFTR promoter.
Targeting cystic fibrosis inflammation in the age of CFTR modulators: focus on macrophages.
Targeting different binding sites in the CFTR structures allows to synergistically potentiate channel activity.
Targeting ENaC as a Molecular Suspect in Cystic Fibrosis.
Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis.
Targeting ion channels in cystic fibrosis.
Targeting miRNA-based medicines to cystic fibrosis airway epithelial cells using nanotechnology.
Targeting Molecular Chaperones for the Treatment of Cystic Fibrosis: Is It a Viable Approach?
Targeting Nonsense: Optimization of 1,2,4-Oxadiazole TRIDs to Rescue CFTR Expression and Functionality in Cystic Fibrosis Cell Model Systems.
Targeting of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Protein with a Technetium-99m Imaging Probe.
Targeting the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Protein for the Treatment of Cystic Fibrosis.
Targeting the Intracellular Environment in Cystic Fibrosis: Restoring Autophagy as a Novel Strategy to Circumvent the CFTR Defect.
Targeting the PI3K/Akt/mTOR signalling pathway in Cystic Fibrosis.
Targets for cystic fibrosis therapy: proteomic analysis and correction of mutant cystic fibrosis transmembrane conductance regulator.
TAS2R38 is a novel modifier gene in patients with 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.
Tear Production After Bilateral Main Lacrimal Gland Resection in Rabbits.
Technical validation of a TM Biosciences Luminex-based multiplex assay for detecting the American College of Medical Genetics recommended cystic fibrosis mutation panel.
Technological advances shed light on left ventricular cardiac disturbances in cystic fibrosis.
Telomere shortening is associated with reduced duodenal HCOFormula secretory but normal gastric acid secretory capacity in aging mice.
Temperature-dependent release of ATP from human erythrocytes: mechanism for the control of local tissue perfusion.
Temporal bone pneumatization in Cystic Fibrosis related to genotype?
Temporal regulation of CFTR expression during ovine lung development: implications for CF gene therapy.
Temporal trends in healthcare resource use and associated costs of patients with cystic fibrosis.
Terminal sialylation is altered in airway cells with impaired CFTR-mediated chloride transport.
Testicular Cancer in a Lung Transplant Patient With Cystic Fibrosis: A Case Report.
Testing and reporting ACMG cystic fibrosis mutation panel results.
Testing for homogeneity of gametic disequilibrium across strata.
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.
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.
Testosterone regulates levels of cystic fibrosis transmembrane regulator, adenylate cyclase, and cAMP in the seminal vesicles of orchidectomized rats.
Tetramethylpyrazine stimulates cystic fibrosis transmembrane conductance regulator-mediated anion secretion in distal colon of rodents.
Tetrodotoxin-sensitive Na+ channels and muscarinic and purinergic receptors identified in human erythroid progenitor cells and red blood cell ghosts.
Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del.
Tezacaftor-ivacaftor use in routine care of adults with cystic fibrosis: a medicine use evaluation.
Tezacaftor/Ivacaftor in Subjects with Cystic Fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR.
TGF-?-induced IL-6 prevents development of acute lung injury in influenza A virus-infected F508del CFTR-heterozygous mice.
TGF-?1 Augments the Apical Membrane Abundance of Lemur Tyrosine Kinase 2 to Inhibit CFTR-Mediated Chloride Transport in Human Bronchial Epithelia.
TGF-beta 1 and Cigarette Smoke Inhibit the Ability of ?2-agonists to Enhance Epithelial Permeability.
Tgf-beta downregulation of distinct chloride channels in cystic fibrosis-affected epithelia.
TGF-beta(1) genotype and accelerated decline in lung function of patients with cystic fibrosis.
TGFbeta down-regulation of the CFTR: a means to limit epithelial chloride secretion.
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.
The 3120 +1G-->A splicing mutation in CFTR is common in Brazilian cystic fibrosis patients.
The 8.1 ancestral MHC haplotype is associated with delayed onset of colonization in 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.
The ?F508 Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator Is Associated With Progressive Insulin Resistance and Decreased Functional ?-Cell Mass in Mice.
The ABC transporter gene family of Daphnia pulex.
The actin filament disrupter cytochalasin D activates the recombinant cystic fibrosis transmembrane conductance regulator Cl- channel in mouse 3T3 fibroblasts.
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.
The adenosine 2A receptor agonist GW328267C improves lung function after acute lung injury in rats.
The adenosine A2B receptor is involved in anion secretion in human pancreatic duct Capan-1 epithelial cells.
The alpha2beta1 isoform of guanylyl cyclase mediates plasma membrane localized nitric oxide signalling.
The altered gut microbiota in adults with cystic fibrosis.
The amiloride-inhibitable Na+ conductance is reduced by the cystic fibrosis transmembrane conductance regulator in normal but not in cystic fibrosis airways.
The amino-terminal cyclic nucleotide binding site of the type II cGMP-dependent protein kinase is essential for full cyclic nucleotide-dependent activation.
The amino-terminal portion of CFTR forms a regulated Cl- channel.
The anion transporter SLC26A9 localizes to tight junctions and is degraded by the proteasome when co-expressed with F508del-CFTR.
The antigen-binding characteristics of mAbs derived from in vivo priming of avian B cells.
The apical anion exchanger Slc26a6 promotes oxalate secretion by murine submandibular gland acinar cells.
The Application of Bicarbonate Recovers the Chemical-Physical Properties of Airway Surface Liquid in Cystic Fibrosis Epithelia Models.
The application of telemedicine in the follow-up of lung transplantation in a patient with cystic fibrosis.
The Arabidopsis thaliana ABC protein superfamily, a complete inventory.
The ATP-binding component of a prokaryotic traffic ATPase is exposed to the periplasmic (external) surface.
The Balance between the Safety of Mother, Fetus, and Newborn Undergoing Cystic Fibrosis Transmembrane Conductance Regulator Treatments during Pregnancy.
The bidirectional relationship between CFTR and lipids.
The bioflavonoid compound, sinupret, stimulates transepithelial chloride transport in vitro and in vivo.
The biogenesis, traffic, and function of the cystic fibrosis transmembrane conductance regulator.
The block of CFTR by scorpion venom is state-dependent.
The buffer capacity of airway epithelial secretions.
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.
The C-terminus of nucleotide binding domain 1 contains critical features for cystic fibrosis transmembrane conductance regulator trafficking and activation.
The calpain, caspase 12, caspase 3 cascade leading to apoptosis is altered in F508del-CFTR expressing cells.
The carboxyl terminus of the cystic fibrosis transmembrane conductance regulator binds to AP-2 clathrin adaptors.
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.
The CF-modifying gene EHF promotes p.Phe508del-CFTR residual function by altering protein glycosylation and trafficking in epithelial cells.
The CFTR and ENaC debate: how important is ENaC in CF lung disease?
The CFTR gene and regulation of its expression.
The CFTR gene variants in Japanese children with idiopathic pancreatitis.
The CFTR ion channel: gating, regulation, and anion permeation.
The CFTR M470V gene variant as a potential modifier of COPD severity: study of Serbian population.
The CFTR M470V, intron 8 poly-T, and 8 TG-repeats detection in Chinese males with congenital bilateral absence of the vas deferens.
The CFTR Met 470 allele is associated with lower birth rates in fertile men from a population isolate.
The CFTR Mutation c.3453G > C (D1152H) Confers an Anion Selectivity Defect in Primary Airway Tissue that Can Be Rescued by Ivacaftor.
The CFTR P67L variant reveals a key role for N-terminal lasso helices in channel folding, maturation, and pharmacologic rescue.
The CFTR polymorphisms poly-T, TG-repeats and M470V in Chinese males with congenital bilateral absence of the vas deferens.
The CFTR-Associated Ligand Arrests the Trafficking of the Mutant ?F508 CFTR Channel in the ER Contributing to Cystic Fibrosis.
The CFTR-derived peptides as a model of sequence-specific protein aggregation.
The CFTR-mediated protein secretion defect: pharmacological correction.
The challenges and promises of new therapies for cystic fibrosis.
The changing face of the exocrine pancreas in cystic fibrosis: pancreatic sufficiency, pancreatitis and genotype.
The chloride channel CFTR controls cellular quiescence by hyperpolarizing the cell membrane during diapause in the crustacean Artemia.
The chloride channel CFTR is not required for cyst growth in an ADPKD mouse model.
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.
The classification of ATP-binding cassette subfamily A member 3 mutations using the cystic fibrosis transmembrane conductance regulator classification system.
The CLIC1 chloride channel is regulated by the cystic fibrosis transmembrane conductance regulator when expressed in Xenopus oocytes.
The Clinical Biology of Cystic Fibrosis Transmembrane Regulator Protein: Its Role and Function in Extrapulmonary Disease.
The cochaperone HspBP1 inhibits the CHIP ubiquitin ligase and stimulates the maturation of the cystic fibrosis transmembrane conductance regulator.
The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment.
The complex relationships between cystic fibrosis and congenital bilateral absence of the vas deferens: clinical, electrophysiological and genetic data.
The course of glucose intolerance in children with cystic fibrosis: a retrospective study - preliminary report.
The cubicon method for concentrating membrane proteins in the cubic mesophase.
The CYSMA web server: An example of integrative tool for in silico analysis of missense variants identified in Mendelian disorders.
The cystic fibrosis conductance regulator gene exon sequence is normal in a patient with edematous eosinophilic nasal polyps.
The cystic fibrosis gene and its product CFTR.
The cystic fibrosis gene: a molecular genetic perspective.
The cystic fibrosis intestine.
The cystic fibrosis mutation (delta F508) does not influence the chloride channel activity of CFTR.
The cystic fibrosis mutation G1349D within the signature motif LSHGH of NBD2 abolishes the activation of CFTR chloride channels by genistein.
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.
The cystic fibrosis of exocrine pancreas.
The cystic fibrosis transmembrane conductance regulator (CFTR) and chloride-dependent ion fluxes of ovine vocal fold epithelium.
The cystic fibrosis transmembrane conductance regulator (CFTR) and its stability.
The cystic fibrosis transmembrane conductance regulator (CFTR) inhibits ENaC through an increase in the intracellular Cl- concentration.
The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in maturation stage ameloblasts, odontoblasts and bone cells.
The cystic fibrosis transmembrane conductance regulator (Cftr) modulates the timing of puberty in mice.
The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Uses its C-Terminus to Regulate the A2B Adenosine Receptor.
The cystic fibrosis transmembrane conductance regulator (CFTR): three-dimensional structure and localization of a channel gate.
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.
The cystic fibrosis transmembrane conductance regulator activates aquaporin 3 in airway epithelial cells.
The cystic fibrosis transmembrane conductance regulator and ATP.
The cystic fibrosis transmembrane conductance regulator and its function in epithelial transport.
The cystic fibrosis transmembrane conductance regulator as a biomarker in non-small cell lung cancer.
The cystic fibrosis transmembrane conductance regulator as a marker of human pancreatic duct development.
The cystic fibrosis transmembrane conductance regulator attenuates the endogenous Ca2+ activated Cl- conductance of Xenopus oocytes.
The cystic fibrosis transmembrane conductance regulator chloride channel. Iodide block and permeation.
The cystic fibrosis transmembrane conductance regulator Cl(-) channel: a versatile engine for transepithelial ion transport.
The cystic fibrosis transmembrane conductance regulator gene and ion channel function in patients with idiopathic pancreatitis.
The cystic fibrosis transmembrane conductance regulator gene.
The cystic fibrosis transmembrane conductance regulator impedes proteolytic stimulation of the epithelial Na+ channel.
The cystic fibrosis transmembrane conductance regulator in reproductive health and disease.
The cystic fibrosis transmembrane conductance regulator interacts with and regulates the activity of the HCO3- salvage transporter human Na+-HCO3- cotransport isoform 3.
The cystic fibrosis transmembrane conductance regulator interacts with multiple Ig domains of filamin A.
The cystic fibrosis transmembrane conductance regulator is a dual ATP and chloride channel.
The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor.
The cystic fibrosis transmembrane conductance regulator is not a base transporter in isolated duodenal epithelial cells.
The cystic fibrosis transmembrane conductance regulator is regulated by a direct interaction with the protein phosphatase 2A.
The cystic fibrosis transmembrane conductance regulator mediates transepithelial fluid secretion by human autosomal dominant polycystic kidney disease epithelium in vitro.
The Cystic Fibrosis Transmembrane Conductance Regulator's Expanding SNARE Interactome.
The cystic fibrosis transmembrane conductance regulator.
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.
The cystic fibrosis transmembrane conductance regulator. Nucleotide binding to a synthetic peptide segment from the second predicted nucleotide binding fold.
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.
The cystic fibrosis transmembrane conductance regulator: an intriguing protein with pleiotropic functions.
The cystic fibrosis transmembrane recruiter the alter ego of CFTR as a multi-kinase anchor.
The cystic fibrosis transmembrane regulator (CFTR) in the kidney.
The cystic fibrosis transmembrane regulator forms macromolecular complexes with PDZ domain scaffold proteins.
The cystic fibrosis transmembrane regulator gene and male infertility.
The cystic fibrosis transmembrane regulator gene is expressed in the human endocervix throughout the menstrual cycle.
The cystic fibrosis transmembrane regulator is present and functional in endosomes. Role as a determinant of endosomal pH.
The cystic fibrosis-causing mutation {Delta}F508 affects multiple steps in CFTR biogenesis.
The cystic-fibrosis-associated {Delta}F508 mutation confers post-transcriptional destabilization on the C. elegans ABC transporter PGP-3.
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.
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.
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.
The Delta F508 mutation shortens the biochemical half-life of plasma membrane CFTR in polarized epithelial cells.
The DeltaF508 mutation disrupts packing of the transmembrane segments of the cystic fibrosis transmembrane conductance regulator.
The deltaF508 mutation in the cystic fibrosis transmembrane conductance regulator alters control of essential fatty acid utilization in epithelial cells.
The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colon.
The detection and significance of cystic fibrosis transmembrane conductance regulator gene promoter mutations in Chinese patients with congenital bilateral absence of the vas deferens.
The deubiquitinating enzyme USP10 regulates the endocytic recycling of CFTR in airway epithelial cells.
The deubiquitinating enzyme USP10 regulates the post-endocytic sorting of CFTR in airway epithelial cells.
The differential role of renoguanylin in osmoregulation and apical Cl-/HCO3- exchange activity in the posterior intestine of the Gulf toadfish (Opsanus beta).
The direct early diagnosis of cystic fibrosis by the detection of the delta F508 CFTR gene mutation in a prematurely delivered boy.
The distribution and expression of CFTR restricts electrogenic anion secretion to the ileum of the brushtail possum, Trichosurus vulpecula.
The duct cell in cystic fibrosis.
The effect of ambroxol on chloride transport, CFTR and ENaC in cystic fibrosis airway epithelial cells.
The effect of chloride concentration on human neutrophil functions: potential relevance to 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.
The Effect of Sodium Bicarbonate, a Beneficial Adjuvant Molecule in Cystic Fibrosis, on Bronchial Epithelial Cells Expressing a Wild-Type or Mutant CFTR Channel.
The Effect of Synonymous Single-Nucleotide Polymorphisms on an Atypical Cystic Fibrosis Clinical Presentation.
The effect of varying tonicity on nasal epithelial ion transport in 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.
The effects of acute salinity challenges on osmoregulation in Mozambique tilapia reared in a tidally changing salinity.
The Effects of the Anti-aging Protein Klotho on Mucociliary Clearance.
The effects of transfer from steady-state to tidally-changing salinities on plasma and branchial osmoregulatory variables in adult Mozambique tilapia.
The endoplasmic reticulum-associated Hsp40 DNAJB12 and Hsc70 cooperate to facilitate RMA1 E3-dependent degradation of nascent CFTRDeltaF508.
The endothelium in hypoxic pulmonary vasoconstriction.
The Enigmatic Gut in Cystic Fibrosis: Linking Inflammation, Dysbiosis, and the Increased Risk of Malignancy.
The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis lung disease.
The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis.
The epithelium as a target for therapy in cystic fibrosis.
The ER-resident ubiquitin-specific protease 19 participates in the UPR and rescues ERAD substrates.
The Etiology and Clinical Course of Chronic Pancreatitis in Children With Early Onset of the Disease.
The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis.
The expression of three topologically distinct membrane proteins elicits unique stress response pathways in the yeast Saccharomyces cerevisiae.
The extracellular calcium-sensing receptor regulates human fetal lung development via CFTR.
The Extrapulmonary Effects of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Cystic Fibrosis.
The F508del mutation in cystic fibrosis transmembrane conductance regulator gene impacts bone formation.
The Fifth Transmembrane Segment of Cystic Fibrosis Transmembrane Conductance Regulator Contributes to Its Anion Permeation Pathway.
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.
The first nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator can function as an active ATPase.
The First Successful Lung Transplantation in a Korean Child with 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.
The function of the intermediate compartment in pre-Golgi trafficking involves its stable connection with the centrosome.
The future of CFTR modulating therapies for cystic fibrosis.
The future of cystic fibrosis care: a global perspective.
The gallbladder and biliary tract in cystic fibrosis.
The gasotransmitter hydrogen sulfide inhibits transepithelial anion secretion of pregnant mouse endometrial epithelium.
The gating of the CFTR channel.
The gene for congenital chloride diarrhea maps close to but is distinct from the gene for cystic fibrosis transmembrane conductance regulator.
The genetic background of osteoporosis in cystic fibrosis: association analysis with polymorphic markers in four candidate genes.
The gliadin-CFTR connection: new perspectives for the treatment of celiac disease.
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.
The H-loop in the second nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator is required for efficient chloride channel closing.
The HDAC inhibitor SAHA does not rescue CFTR membrane expression in Cystic Fibrosis.
The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis.
The hsa-miR-125a/hsa-let-7e/hsa-miR-99b cluster is potentially implicated in Cystic Fibrosis pathogenesis.
The human CFTR protein expressed in CHO cells activates aquaporin-3 in a cAMP-dependent pathway: study by digital holographic microscopy.
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.
The hypertonic environment differentially regulates wild-type CFTR and TNR-CFTR chloride channels.
The ichthyotoxic alga Chattonella marina induces Na+, K+ -ATPase, and CFTR proteins expression in fish gill chloride cells in vivo.
The impact of CFTR modulator therapies on CF airway microbiology.
The impact of Cystic Fibrosis Transmembrane Regulator Disruption on cardiac function and stress response.
The impact of ivacaftor on sinonasal pathology in S1251N-mediated cystic fibrosis patients.
The impact of personalised therapies on respiratory medicine.
The Impact of Secondhand Smoke Exposure on Children with Cystic Fibrosis: A Review.
The Impact of the CFTR Gene Discovery on Cystic Fibrosis Diagnosis, Counseling, and Preventive Therapy.
The Impact on Genetic Testing of Mutational Patterns of CFTR Gene in Different Clinical Macrocategories of Cystic Fibrosis.
The impacts of along-channel acupuncture on the protein expressions of the chloride channel of the rats with myocardial ischemia.
The implications and management of cystic fibrosis screen positive, inconclusive diagnosis patients.
The implications of CFTR structural studies for cystic fibrosis drug development.
The inhibition mechanism of non-phosphorylated Ser768 in the regulatory domain of cystic fibrosis transmembrane conductance regulator.
The inhibitory role of purinergic P2Y receptor on Mg2+ transport across intestinal epithelium-like Caco-2 monolayer.
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.
The interaction between syntaxin 1A and cystic fibrosis transmembrane conductance regulator Cl- channels is mechanistically distinct from syntaxin 1A-SNARE interactions.
The interaction of gut microbes with host ABC transporters.
The intestinal guanylin system and seawater adaptation in eels.
The Intestinal Microbiome and Cystic Fibrosis Transmembrane Conductance Regulator Modulators: Emerging Themes in the Management of Gastrointestinal Manifestations of Cystic Fibrosis.
The investigational Cystic Fibrosis drug Trimethylangelicin directly modulates CFTR by stabilizing the first membrane-spanning domain.
The IP3 R Binding Protein Released With Inositol 1,4,5-Trisphosphate Is Expressed in Rodent Reproductive Tissue and Spermatozoa.
The KDEL receptor has a role in the biogenesis and trafficking of the epithelial sodium channel (ENaC).
The L441P mutation of cystic fibrosis transmembrane conductance regulator and its molecular pathogenic mechanisms in a Korean patient with cystic fibrosis.
The lactoperoxidase system links anion transport to host defense in cystic fibrosis.
The lamellae-free-type pseudobranch of the euryhaline milkfish (Chanos chanos) is a Na(+), K(+)-ATPase-abundant organ involved in hypoosmoregulation.
The low PLC-?1 expression in cystic fibrosis bronchial epithelial cells induces upregulation of TRPV6 channel activity.
The mechanism of cystic fibrosis transmembrane conductance regulator transcriptional repression during the unfolded protein response.
The mechanism underlying cystic fibrosis transmembrane conductance regulator transport from the endoplasmic reticulum to the proteasome includes Sec61beta and a cytosolic, deglycosylated intermediary.
The Mechanistic Links between Insulin and Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Cl(-) Channel.
The membrane-bound bile acid receptor TGR5 is localized in the epithelium of human gallbladders.
The MglA component of the binding protein-dependent galactose transport system of Salmonella typhimurium is a galactose-stimulated ATPase.
The Microbiome in Cystic Fibrosis Pulmonary Disease.
The mitochondrial Complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function.
The molecular basis for disease variability in cystic fibrosis.
The molecular basis of partial penetrance of splicing mutations in cystic fibrosis.
The molecular biology of chloride channels.
The molecular biology of nasal polyposis.
The molecular chaperone Hsc70 assists the in vitro folding of the N-terminal nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator.
The molecular mechanism of CFTR- and secretin-dependent renal bicarbonate excretion.
The most common cystic fibrosis-associated mutation destabilizes the dimeric state of the nucleotide-binding domains of CFTR.
The multi-herbal drug STW 5 (Iberogast(R)) has prosecretory action in the human intestine.
The Multifaceted Roles of MicroRNAs in Cystic Fibrosis.
The mycotoxin ochratoxin A alters intestinal barrier and absorption functions but has no effect on chloride secretion.
The Na(+)/H(+) exchanger regulatory factor 2 mediates phosphorylation of serum- and glucocorticoid-induced protein kinase 1 by 3-phosphoinositide-dependent protein kinase 1.
The necessity of complete CFTR mutational analysis of an infertile couple before in vitro fertilization.
The need for vigilance: the case of a false-negative newborn screen for cystic fibrosis.
The neglected biliary mucus and its phosphatidylcholine content: a putative player in pathogenesis of primary cholangitis-a narrative review article.
The new frontier: gene and oligonucleotide therapy.
The non-hydrolytic pathway of cystic fibrosis transmembrane conductance regulator ion channel gating.
The Novel CFTR Mutation A457P in a Male with a Delayed Diagnosis of Cystic Fibrosis.
The novel complex allele [A238V;F508del] of the CFTR gene: clinical phenotype and possible implications for cystic fibrosis etiological therapies.
The nucleotide binding folds of the cystic fibrosis transmembrane conductance regulator are extracellularly accessible.
The occurrence of various non-delta F508 CFTR gene mutations among Hungarian cystic fibrosis patients.
The odorant receptor OR2W3 on airway smooth muscle evokes bronchodilation via a cooperative chemosensory tradeoff between TMEM16A and CFTR.
The optimal approach to nutrition and cystic fibrosis: latest evidence and recommendations.
The origin of and subcellular mechanisms causing pancreatic bicarbonate secretion.
The osmoregulatory effects of rearing Mozambique tilapia in a tidally changing salinity.
The pancreatitis-associated protein (PAP). A new candidate for neonatal screening of cystic fibrosis.
The patch-clamp and planar lipid bilayer techniques: powerful and versatile tools to investigate the CFTR Cl- channel.
The PDZ-interacting domain of cystic fibrosis transmembrane conductance regulator is required for functional expression in the apical plasma membrane.
The PEST sequence does not contribute to the stability of the cystic fibrosis transmembrane conductance regulator.
The phenotypic consequences of CFTR mutations.
The phorbol ester PMA and cyclic AMP activate different Cl(-) and HCO3(-) fluxes in C127 cells expressing CFTR.
The Phosphodiesterase Inhibitor Ensifentrine Reduces Production of Proinflammatory Mediators in Well Differentiated Bronchial Epithelial Cells by Inhibiting PDE4.
The phospholipid flippase ATP8B1 mediates apical localization of the cystic fibrosis transmembrane regulator.
The Physiology and Pathophysiology of Pancreatic Ductal Secretion: The Background for Clinicians.
The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation.
The porcine lung as a potential model for cystic fibrosis.
The pore architecture of the cystic fibrosis transmembrane conductance regulator channel revealed by co-mutation in pore-forming transmembrane regions.
The possible additional role of the cystic fibrosis transmembrane regulator to motoneuron inhibition produced by glycine effects.
The Potential Causes of Cystic Fibrosis-Related Diabetes.
The Potential of Wharton's Jelly Derived Mesenchymal Stem Cells in Treating Patients with Cystic Fibrosis.
The Potential Role and Regulatory Mechanisms of MUC5AC in Chronic Obstructive Pulmonary Disease.
The power stroke driven by ATP binding in CFTR as studied by molecular dynamics simulations.
The preclinical discovery and development of the combination of ivacaftor + tezacaftor used to treat cystic fibrosis.
The predictive potential of the sweat chloride test in cystic fibrosis patients with the G551D mutation.
The Probable, Possible, and Novel Functions of ERp29.
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.
The protein kinase A-regulated cardiac Cl- channel resembles the cystic fibrosis transmembrane conductance regulator.
The protein tyrosine kinase pathway is not involved in the regulation of K+ transport across the rat colon.
The proteome speciation of an immortalized cystic fibrosis cell line: New perspectives on the pathophysiology of the disease.
The Pseudomonas aeruginosa secreted protein PA2934 decreases apical membrane expression of the cystic fibrosis transmembrane conductance regulator.
The rad50 signature motif: essential to ATP binding and biological function.
The regulatory role of vasoactive intestinal peptide in lacrimal gland ductal fluid secretion: A new piece of the puzzle in tear production.
The relationship between cell proliferation, Cl- secretion, and renal cyst growth: a study using CFTR inhibitors.
The relationship between genotype and exercise tolerance in children with cystic fibrosis.
The relationship between genotype and phenotype in cystic fibrosis.
The relationship of chronic mucin secretion to airway disease in normal and CFTR-deficient mice.
The relative binding affinities of PDZ partners for CFTR: a biochemical basis for efficient endocytic recycling.
The relevance of sweat testing for the diagnosis of cystic fibrosis in the genomic era.
The replication timing of CFTR and adjacent genes.
The role of CDX2 in renal tubular lesions during diabetic kidney disease.
The role of chloride anion and CFTR in killing of Pseudomonas aeruginosa by normal and CF neutrophils.
The role of common single-nucleotide polymorphisms on exon 9 and exon 12 skipping in nonmutated CFTR alleles.
The role of cystic fibrosis transmembrane conductance regulator chloride channel in beta-receptor-mediated regulation of alveolar fluid clearance.
The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating.
The role of endothelial cells in cystic fibrosis.
The role of epithelial P2Y2 and P2Y4 receptors in the regulation of intestinal chloride secretion.
The role of inflammation in the pathophysiology of CF lung disease.
The role of local angiotensins and prostaglandins in the control of anion secretion by the rat epididymis.
The role of nuclear factor E2-Related factor 2 and uncoupling protein 2 in glutathione metabolism: Evidence from an in vivo gene knockout study.
The role of pancreatic ducts in the pathogenesis of acute pancreatitis.
The role of regulated CFTR trafficking in epithelial secretion.
The role of SLC9A3 in Taiwanese patients with congenital bilateral absence of vas deferens (CBAVD).
The Role of Specialized Pro-Resolving Mediators in Cystic Fibrosis Airways Disease.
The role of sphingolipids and ceramide in pulmonary inflammation in 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.
The role of the F508C mutation in congenital bilateral absence of the vas deferens.
The ROMK-cystic fibrosis transmembrane conductance regulator connection: new insights into the relationship between ROMK and cystic fibrosis transmembrane conductance regulator channels.
The safety and efficacy of oral docosahexaenoic acid supplementation for the treatment of primary sclerosing cholangitis - a pilot study.
The safety of lumacaftor and ivacaftor for the treatment of cystic fibrosis.
The SbcCD protein of Escherichia coli is related to two putative nucleases in the UvrA superfamily of nucleotide-binding proteins.
The search for susceptibility genes of COPD.
The second half of the cystic fibrosis transmembrane conductance regulator forms a functional chloride channel.
The self-interaction of native TDP-43 C terminus inhibits its degradation and contributes to early proteinopathies.
THE SHARK RECTAL GLAND MODEL: A CHAMPION OF RECEPTOR MEDIATED CHLORIDE SECRETION THROUGH CFTR.
The sheep genome contributes to localization of control elements in a human gene with complex regulatory mechanisms.
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.
The short-term effects of ORKAMBI (lumacaftor/ivacaftor) on regional and distal lung structures using functional respiratory imaging.
The silent codon change I507-ATC->ATT contributes to the severity of the ?F508 CFTR channel dysfunction.
The SLC26 gene family of anion transporters and channels.
The SLC26 gene family of multifunctional anion exchangers.
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.
The Small GTPase Rab11b Regulates Degradation of Surface Membrane L-Type Cav1.2 Channels.
The spectrum of CFTR mutations in south-west German cystic fibrosis patients.
The spectrum of CFTR-related disease.
The study of cystic fibrosis transmembrane conductance regulator gene mutations in a group of patients from Romania.
The substituted benzimidazolone NS004 is an opener of the cystic fibrosis chloride channel.
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.
The susceptibility of T5-TG12 of the CFTR gene in chronic bronchitis occurrence in a Chinese population in Jiangsu province, China.
The Sweat Metabolome of Screen-Positive Cystic Fibrosis Infants: Revealing Mechanisms beyond Impaired Chloride Transport.
The swelling-activated anion conductance in the mouse renal inner medullary collecting duct cell line mIMCD-K2.
The TAK1?IKK??TPL2?MKK1/MKK2 Signaling Cascade Regulates IL-33 Expression in Cystic Fibrosis Airway Epithelial Cells Following Infection by Pseudomonas aeruginosa.
The testis anion transporter TAT1 (SLC26A8) physically and functionally interacts with the cystic fibrosis transmembrane conductance regulator channel: a potential role during sperm capacitation.
The therapeutic potential of the human cystic fibrosis transmembrane conductance regulator gene.
The transmembrane protein CBP plays a role in transiently anchoring small clusters of Thy-1, a GPI-anchored protein, to the cytoskeleton.
The Two ATP Binding Sites of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Play Distinct Roles in Gating Kinetics and Energetics.
The two halves of CFTR form a dual-pore ion channel.
The two nucleotide-binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) have distinct functions in controlling channel activity.
The type IVB pili of Salmonella enterica serovar Typhi bind to the cystic fibrosis transmembrane conductance regulator.
The tyrosine kinase p60c-src regulates the fast gate of the cystic fibrosis transmembrane conductance regulator chloride channel.
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.
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.
The uroguanylin gene (Guca1b) is linked to guanylin (Guca2) on mouse chromosome 4.
The use of DHPLC (Denaturing High Performance Liquid Chromatography) in II level screening of the CFTR gene in Prenatal Diagnosis.
The use of ivacaftor in CFTR mutations resulting in residual functioning protein.
The use of lumacaftor/ivacaftor to treat acute deterioration in paediatric cystic fibrosis.
The winged helix transcription factor HFH-4 is expressed during choroid plexus epithelial development in the mouse embryo.
The yin and yang of cystic fibrosis transmembrane conductance regulator function: implications for chronic lung disease.
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.
The {Delta}F508 Mutation Causes CFTR Misprocessing and Cystic Fibrosis-Like Disease in Pigs.
Therapeutic Approaches to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis.
Therapeutic approaches to CFTR dysfunction: From discovery to drug development.
Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting.
Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation.
Therapeutic challenges posed by critical drug-drug interactions in cystic fibrosis.
Therapeutic peptides for the treatment of cystic fibrosis: Challenges and perspectives.
Therapeutic pipeline for individuals with cystic fibrosis with mutations nonresponsive to current cystic fibrosis transmembrane conductance regulator modulators.
Therapeutic Potential for CFTR Correctors in Autosomal Recessive Polycystic Kidney Disease.
Therapeutic potential of cystic fibrosis transmembrane conductance regulator (CFTR) inhibitors in polycystic kidney disease.
Therapeutic potential of serum and glucocorticoid inducible kinase inhibition.
Therapeutic strategies to correct malfunction of CFTR.
Therapeutically Targeting Tumor Necrosis Factor-?/Sphingosine-1-Phosphate Signaling Corrects Myogenic Reactivity in Subarachnoid Hemorrhage.
Therapies directed at the basic defect in cystic fibrosis.
Theratyping cystic fibrosis in vitro in ALI-culture and organoid models generated from patient-derived nasal epithelial Conditionally Reprogrammed Stem Cells.
Thermal instability of ?F508 cystic fibrosis transmembrane conductance regulator (CFTR) channel function: protection by single suppressor mutations and inhibiting channel activity.
Thermodynamic study of the native and phosphorylated regulatory domain of the CFTR.
Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion.
Thiazolidinone CFTR inhibitors with improved water solubility identified by structure-activity analysis.
Thiocyanate as a probe of the cystic fibrosis transmembrane conductance regulator chloride channel pore.
Thiocyanate transport in resting and IL-4-stimulated human bronchial epithelial cells: role of pendrin and anion channels.
Thixotropic solutions enhance viral-mediated gene transfer to airway epithelia.
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.
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.
Three point mutations in the CFTR gene in French cystic fibrosis patients: identification by denaturing gradient gel electrophoresis.
Three-dimensional reconstruction of human cystic fibrosis transmembrane conductance regulator chloride channel revealed an ellipsoidal structure with orifices beneath the putative transmembrane domain.
Thymosin ?-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia.
Thymosin ?1 represents a potential potent single-molecule-based therapy for cystic fibrosis.
Thymosin alpha 1 exerts beneficial extrapulmonary effects in cystic fibrosis.
Thyroid iodide efflux: a team effort?
Time course of salinity adaptation in a strongly euryhaline estuarine teleost, fundulus heteroclitus: a multivariable approach
Time for a gut check: Pancreatic sufficiency resulting from CFTR modulator use.
Timing and sequence of differentiation of embryonic rat hepatocytes along the biliary epithelial lineage.
Tissue-specific in vivo transcription start sites of the human and murine cystic fibrosis genes.
Tissue-specific modulation of glucocorticoid receptor expression in response to salinity acclimation in rainbow trout.
TMEM16A chloride channel does not drive mucus production.
TMEM16A drives renal cyst growth by augmenting Ca2+ signaling in M1 cells.
TMEM16A in Cystic Fibrosis: Activating or Inhibiting?
TMEM16A: An Alternative Approach to Restoring Airway Anion Secretion in Cystic Fibrosis?
Tobacco carcinogen NNK transporter MRP2 regulates CFTR function in lung epithelia: implications for lung cancer.
Tolbutamide causes open channel blockade of cystic fibrosis transmembrane conductance regulator Cl- channels.
Toll-like receptor-4 genotype influences the survival of cystic fibrosis mice.
Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.
Topogenesis of cystic fibrosis transmembrane conductance regulator (CFTR): regulation by the amino terminal transmembrane sequences.
Topological model of membrane domain of the cystic fibrosis transmembrane conductance regulator.
TorsinA participates in endoplasmic reticulum-associated degradation.
Toward an animal model of cystic fibrosis: targeted interruption of exon 10 of the cystic fibrosis transmembrane regulator gene in embryonic stem cells.
Toward the pharmacogenomics of cystic fibrosis--an update.
Towards gene therapy of cystic fibrosis.
Towards next generation therapies for cystic fibrosis: Folding, function and pharmacology of CFTR.
Tracking of quantum dot-labeled CFTR shows near immobilization by C-terminal PDZ interactions.
Traditional Chinese formula, lubricating gut pill, improves loperamide-induced rat constipation involved in enhance of Cl- secretion across distal colonic epithelium.
Traffic pattern of cystic fibrosis transmembrane regulator through the early exocytic pathway.
Traffic-independent function of the Sar1p/COPII machinery in proteasomal sorting of the cystic fibrosis transmembrane conductance regulator.
Trafficking and Function of the Cystic Fibrosis Transmembrane Conductance Regulator: A Complex Network of Post-Translational Modifications.
Trafficking of GFP-tagged DeltaF508-CFTR to the plasma membrane in a polarized epithelial cell line.
Trans-complementation of E1-deleted adenovirus: a new vector to reduce the possibility of codissemination of wild-type and recombinant adenoviruses.
Transcellular thiocyanate transport by human airway epithelia.
Transcript analysis of CFTR frameshift mutations in lymphocytes using the reverse transcription-polymerase chain reaction technique and the protein truncation test.
Transcription of cystic fibrosis transmembrane conductance regulator requires a CCAAT-like element for both basal and cAMP-mediated regulation.
Transcriptional adaptation to cystic fibrosis transmembrane conductance regulator deficiency.
Transcriptional analysis of cystic fibrosis airways at single-cell resolution reveals altered epithelial cell states and composition.
Transcriptional regulation of CFTR gene expression.
Transcriptional repression of the cystic fibrosis transmembrane conductance regulator gene, mediated by CCAAT displacement protein/cut homolog, is associated with histone deacetylation.
Transcriptional targeting in the airway using novel gene regulatory elements.
Transcriptome Profiling and Molecular Therapeutic Advances in Cystic Fibrosis: Recent Insights.
Transcriptomic analysis of CFTR-impaired endothelial cells reveals a pro-inflammatory phenotype.
Transcriptomic responses to Ivacaftor and prediction of Ivacaftor clinical responsiveness.
Transcytosis maintains CFTR apical polarity in the face of constitutive and mutation-induced basolateral missorting.
Transduction of Surface and Basal Cells in Rhesus Macaque Lung Following Repeat Dosing with AAV1CFTR.
Transepithelial bicarbonate secretion: lessons from the pancreas.
Transepithelial electrical measurements with the Ussing chamber.
Transepithelial fluctuation analysis of chloride secretion.
Transepithelial Fluid and Salt Re-Absorption Regulated by cGK2 Signals.
Transepithelial ion transport is suppressed in hypoxic sinonasal epithelium.
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.
Transfection of wild-type CFTR into cystic fibrosis lymphocytes restores chloride conductance at G1 of the cell cycle.
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.
Transfer of the Cystic Fibrosis Transmembrane Conductance Regulator to Human Cystic Fibrosis Cells Mediated by Extracellular Vesicles.
Transformative therapies for rare CFTR missense alleles.
Transforming growth factor beta1 impairs CFTR-mediated anion secretion across cultured porcine vas deferens epithelial monolayer via the p38 MAPK pathway.
Transforming growth factor beta1 inhibits cystic fibrosis transmembrane conductance regulator-dependent cAMP-stimulated alveolar epithelial fluid transport via a phosphatidylinositol 3-kinase-dependent mechanism.
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.
Transient anchorage of cross-linked glycosyl-phosphatidylinositol-anchored proteins depends on cholesterol, Src family kinases, caveolin, and phosphoinositides.
Transient gene expression from yeast artificial chromosome DNA in mammalian cells is enhanced by adenovirus.
Transient in utero disruption of cystic fibrosis transmembrane conductance regulator causes phenotypic changes in alveolar type II cells in adult rats.
Transient transfection of polarized epithelial monolayers with CFTR and reporter genes using efficacious lipids.
Transimmortalized mouse intestinal cells (m-ICc12) that maintain a crypt phenotype.
Transmembrane domain of cystic fibrosis transmembrane conductance regulator: design, characterization, and secondary structure of synthetic peptides m1-m6.
Transmembrane helical interactions in the CFTR channel pore.
TRANSMEMBRANE PROTEIN 16A (TMEM16A) IS A CA++ REGULATED CL- SECRETORY CHANNEL IN MOUSE AIRWAYS.
Transmembrane transport of endo- and xenobiotics by mammalian ATP-binding cassette multidrug resistance proteins.
Transmission of male infertility and intracytoplasmic sperm injection (mini-review).
Transmission ratio distortion and maternal effects confound the analysis of modulators of cystic fibrosis disease severity on 19q13.
Transport and function of chloride in vascular smooth muscles.
Transport rates of GABA transporters: regulation by the N-terminal domain and syntaxin 1A.
Treating the Airway Consequences of Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction.
Treating the Underlying Cystic Fibrosis Transmembrane Conductance Regulator Defect in Patients with Cystic Fibrosis.
Treatment of Cystic Fibrosis Patients Homozygous for F508del with Lumacaftor-Ivacaftor (Orkambi®) Restores Defective CFTR Channel Function in Circulating Mononuclear Cells.
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.
Treatment Options for cystic fibrosis: State of the Art and Future Perspectives.
Trichomonas vaginalis infection impairs anion secretion in vaginal epithelium.
TRIM44 links the UPS to SQSTM1/p62-dependent aggrephagy and removing misfolded proteins.
Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells.
Trimethylangelicin reduces IL-8 transcription and potentiates CFTR function.
Triple combination cystic fibrosis transmembrane conductance regulator modulator therapy in the real world - opportunities and challenges.
Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes.
Triplet CFTR modulators: future prospects for treatment of cystic fibrosis.
TRPC6 channel translocation into phagosomal membrane augments phagosomal function.
TRPC6 Links Ca2+ Mishandling to CFTR Channel Dysfunction in Cystic Fibrosis.
Truncating Mutations in the Adhesion G Protein-Coupled Receptor G2 Gene ADGRG2 Cause an X-Linked Congenital Bilateral Absence of Vas Deferens.
Tumor necrosis factor modulation of expression of the cystic fibrosis transmembrane conductance regulator gene.
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.
Tuning of CFTR chloride channel function by location of positive charges within the pore.
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.
Twenty-five years of airway research: personal thoughts.
Two CFTR mutations within codon 970 differently impact on the chloride channel functionality.
Two cystic fibrosis transmembrane conductance regulator mutations have different effects on both pulmonary phenotype and regulation of outwardly rectified chloride currents.
Two mechanisms of genistein inhibition of cystic fibrosis transmembrane conductance regulator Cl- channels expressed in murine cell line.
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.
Two new mutations detected by single-strand conformation polymorphism analysis in cystic fibrosis from Russia.
Two novel and correlated CF-causing insertions in the (TG)mTn tract of the CFTR gene.
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.
Two salt bridges differentially contribute to the maintenance of cystic fibrosis transmembrane conductance regulator (CFTR) channel function.
Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.
Two years of newborn screening for cystic fibrosis in Turkey: Çukurova experience.
Two-pore-domain potassium channels support anion secretion from human airway Calu-3 epithelial cells.
Type I, II, III, IV, and V cystic fibrosis transmembrane conductance regulator defects and opportunities for therapy.
Type II protein kinase A regulates CFTR in airway, pancreatic, and intestinal cells.
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.
Tyrosine kinase c-Src constitutes a bridge between cystic fibrosis transmembrane regulator channel failure and MUC1 overexpression in cystic fibrosis.
Tyrosine kinase-independent extracellular action of genistein on the CFTR Cl- channel in guinea pig ventricular myocytes and CFTR-transfected mouse fibroblasts.
Tyrosine phosphorylation is a novel pathway for regulation of chloride secretion in shark rectal gland.
Ubiquitin C-terminal hydrolase-L1 protects cystic fibrosis transmembrane conductance regulator from early stages of proteasomal degradation.
Ubiquitin-mediated proteasomal degradation of ABC transporters: a new aspect of genetic polymorphisms and clinical impacts.
Ubiquitination and degradation of CFTR by the E3 ubiquitin ligase MARCH2 through its association with adaptor proteins CAL and STX6.
Ubiquitination of disease-causing CFTR variants in a microsome-based assay.
UBXD1 is a VCP-interacting protein that is involved in ER-associated degradation.
Ultrastructural lesions in the small bowel of patients with cystic fibrosis.
Ultrastructural localization of variant forms of cystic fibrosis transmembrane conductance regulator in human bronchial epithelial of xenografts.
Unconventional secretion of transmembrane proteins.
Understanding childhood diabetes mellitus: new pathophysiological aspects.
Understanding how cystic fibrosis mutations cause a loss of Cl- channel function.
Understanding how cystic fibrosis mutations disrupt CFTR function: From single molecules to animal models.
Understanding human disease mutations through the use of interspecific genetic variation.
Understanding protein kinase CK2 mis-regulation upon F508del CFTR expression.
Understanding the population structure of North American patients with cystic fibrosis.
Unexpected inactivation of acceptor consensus splice sequence by a -3 C to T transition in intron 2 of the CFTR gene.
Unique microbial communities persist in individual cystic fibrosis patients throughout a clinical exacerbation.
Unique mutations of the cystic fibrosis transmembrane conductance regulator gene of three cases of cystic fibrosis in Nagasaki, Japan.
Unique presentations and chronic complications in adult cystic fibrosis: do they teach us anything about CFTR?
Unitary chloride channels activated by protein kinase C in guinea pig ventricular myocytes.
Unmasking catamenial hemoptysis in the era of CFTR modulator therapy.
Unplugging Mucus in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Unraveling monogenic channelopathies and their implications for complex polygenic disease.
Unraveling the complex genetic model for cystic fibrosis: pleiotropic effects of modifier genes on early cystic fibrosis-related morbidities.
Unravelling the Regions of Mutant F508del-CFTR More Susceptible to the Action of Four Cystic Fibrosis Correctors.
Unusual Cystic Fibrosis Transmembrane Conductance Regulator Mutations and Liver Disease: A Case Series and Review of the Literature.
Unveiling the degradative route of the V247M ?-sarcoglycan mutant responsible for LGMD-2D.
Up-regulated cystic fibrosis transmembrane conductance regulator after anal stenosis in rats.
Up-regulation of acid-gated Na(+) channels (ASICs) by cystic fibrosis transmembrane conductance regulator co-expression in Xenopus oocytes.
Up-regulation of AMP-activated Kinase by Dysfunctional Cystic Fibrosis Transmembrane Conductance Regulator in Cystic Fibrosis Airway Epithelial Cells Mitigates Excessive Inflammation.
Update on Calcium Signaling in Cystic Fibrosis Lung Disease.
Update on clinical trials in the treatment of pulmonary disease in patients with cystic fibrosis.
Update on clinical trials of cystic fibrosis.
Update on new pulmonary therapies.
Update on Respiratory Fungal Infections in Cystic Fibrosis Lung Disease and after Lung Transplantation.
Update on SLC26A3 mutations in congenital chloride diarrhea.
Update on the cellular and molecular aspects of cystic fibrosis transmembrane conductance regulator (CFTR) and male fertility.
Upregulation of CFTR expression but not SLC26A3 and SLC9A3 in ulcerative colitis.
Upregulation of CFTR in patients with endometriosis and its involvement in NF?B-uPAR dependent cell migration.
Upregulation of CFTR Protects against Palmitate-Induced Endothelial Dysfunction by Enhancing Autophagic Flux.
Upregulation of cystic fibrosis transmembrane conductance regulator expression by oestrogen and Bak Foong Pill in mouse uteri.
Uptake of fluorescent dyes associated with the functional expression of the cystic fibrosis transmembrane conductance regulator in epithelial cells.
Urinary Exosomes of Patients with Cystic Fibrosis Unravel CFTR-Related Renal Disease.
Uroguanylin and guanylate cyclase C in the human pancreas: expression and mutuality of ligand/receptor localization as indicators of intercellular paracrine signaling pathways.
Ursodeoxycholate modulates bile flow and bile salt pool independently from the cystic fibrosis transmembrane regulator (Cftr)in mice.
Ursodeoxycholic acid inhibits ENaC and Na/K pump activity to restore airway surface liquid height in cystic fibrosis bronchial epithelial cells.
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.
Use of a membrane potential-sensitive probe to assess biological expression of the cystic fibrosis transmembrane conductance regulator.
Use of nasal potential difference and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis.
Use of protein repair therapy in the treatment of cystic fibrosis.
Use of sodium butyrate to enhance production of retroviral vectors expressing CFTR cDNA.
Utilization of Liquid Chromatography Mass Spectrometry Analyses to Identify LKB1-APC Interaction in Modulating Wnt/?-Catenin Pathway of Lung Cancer Cells.
Validation of double gradient denaturing gradient gel electrophoresis through multigenic retrospective analysis.
Validation of high-resolution DNA melting analysis for mutation scanning of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis.
Validity and Reliability of a Novel Multimodal Questionnaire for the Assessment of Abdominal Symptoms in People with Cystic Fibrosis (CFAbd-Score).
VAMP-associated Proteins (VAP) as Receptors That Couple Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Proteostasis with Lipid Homeostasis.
Vardenafil increases intracellular accumulation of the most prevalent mutant cystic fibrosis transmembrane conductance regulator (CTFR) in human bronchial epithelial cells.
Variable deletion of exon 9 coding sequences in cystic fibrosis transmembrane conductance regulator gene mRNA transcripts in normal bronchial epithelium.
Variable levels of normal RNA in different fetal organs carrying a cystic fibrosis transmembrane conductance regulator splicing mutation.
Variable penetrance and expressivity of the splice altering 5T sequence in the cystic fibrosis gene.
Variable reactivity of an engineered cysteine at position 338 in cystic fibrosis transmembrane conductance regulator reflects different chemical states of the thiol.
Variable Responses to CFTR Correctors in vitro: Estimating the Design Effect in Precision Medicine.
Variant cystic fibrosis phenotypes in the absence of CFTR mutations.
Variants in CFTR untranslated regions are associated with congenital bilateral absence of the vas deferens.
Variants in mannose-binding lectin and tumour necrosis factor alpha affect survival in cystic fibrosis.
Variants in the glutamate-cysteine-ligase gene are associated with cystic fibrosis lung disease.
Variants in the interleukin 8 gene and the response to inhaled bronchodilators in cystic fibrosis.
Variation in a repeat sequence determines whether a common variant of the cystic fibrosis transmembrane conductance regulator gene is pathogenic or benign.
Variation in branchial expression among insulin-like growth-factor binding proteins (igfbps) during Atlantic salmon smoltification and seawater exposure.
Variations in the expression of vasotocin and isotocin receptor genes in the gilthead sea bream Sparus aurata during different osmotic challenges.
Variations of the CFTR gene in the Hanoi-Vietnamese.
Varied clinical significance of ATP-binding cassette C sub-family members for lung adenocarcinoma.
Vasoactive Intestinal Peptide Derived From Liver Mesenchymal Cells Mediates Tight Junction Assembly in Mouse Intrahepatic Bile Ducts.
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.
Vasoactive intestinal peptide regulates sinonasal mucociliary clearance and synergizes with histamine in stimulating sinonasal fluid secretion.
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.
Vasopressin and cAMP stimulate electrogenic chloride secretion in an IMCD cell line.
Vasopressin regulates the phosphorylation state of AMP-activated protein kinase (AMPK) in MDCK-C7 cells.
Vasopressin stimulates long-term net chloride secretion in cortical collecting duct cells.
Vasopressin-stimulated chloride transport in transimmortalized mouse cell lines derived from the distal convoluted tubule and cortical and inner medullary collecting ducts.
VCP/p97 AAA-ATPase does not interact with the endogenous wild-type cystic fibrosis transmembrane conductance regulator.
Vector-specific complementation profiles of two independent primary defects in cystic fibrosis airways.
Vesicular targeting and the control of ion secretion in epithelial cells: implications for cystic fibrosis.
Vibrio parahaemolyticus induces inflammation-associated fluid accumulation via activation of the cystic fibrosis transmembrane conductance regulator.
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.
Visualization of oligonucleotide probes and point mutations in interphase nuclei and DNA fibers using rolling circle DNA amplification.
Visualization of single proteins from stripped native cell membranes: a protocol for high-resolution atomic force microscopy.
Vitamin C controls the cystic fibrosis transmembrane conductance regulator chloride channel.
Vocal Fold Surface Hydration: A Review.
Voltage-dependent block of the cystic fibrosis transmembrane conductance regulator Cl- channel by two closely related arylaminobenzoates.
Voltage-dependent calcium and chloride currents in S17 bone marrow stromal cell line.
Voltage-dependent flickery block of an open cystic fibrosis transmembrane conductance regulator (CFTR) channel pore.
Voltage-dependent gating of the cystic fibrosis transmembrane conductance regulator Cl- channel.
Voltage-sensitive gating induced by a mutation in the fifth transmembrane domain of CFTR.
Volume regulation in cortical collecting duct cells: role of AQP2.
Volume-activated chloride current is not related to P-glycoprotein overexpression.
Volume-regulated chloride channels associated with the human multidrug-resistance P-glycoprotein.
VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle.
VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1.
Walker mutations reveal loose relationship between catalytic and channel-gating activities of purified CFTR (cystic fibrosis transmembrane conductance regulator).
Water Transport Mediated by Other Membrane Proteins.
What is the evidence for genetics in chronic rhinosinusitis?
What is the role of cystic fibrosis transmembrane conductance regulator dysfunction in primary sclerosing cholangitis?
What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?
What shall we do with the damaged proteins in lung disease? Ask the proteasome!
What we know and what we do not know about cystic fibrosis transmembrane conductance regulator.
Wheat bran components modulate intestinal bacteria and gene expression of barrier function relevant proteins in a piglet model.
Whole cell Cl- conductances in mouse choroid plexus epithelial cells do not require CFTR expression.
Whole-blood transcriptomic responses to lumacaftor/ivacaftor therapy in cystic fibrosis.
Whole-Exome Sequencing Identified CFTR Variants in Two Consanguineous Families in China.
Why Mouse Airway Submucosal Gland Serous Cells Do Not Secrete Fluid in Response to cAMP Stimulation.
Widespread airway distribution and short-term phenotypic correction of cystic fibrosis pigs following aerosol delivery of piggyBac/adenovirus.
Widespread and efficient marker gene expression in the airway epithelia of fetal sheep after minimally invasive tracheal application of recombinant adenovirus in utero.
WNK1 and p38-MAPK distribution in ionocytes and accessory cells of euryhaline teleost fish implies ionoregulatory function.
WNK1 and WNK4 modulate CFTR activity.
Worldwide genetic analysis of the CFTR region.
X chromosome transmission ratio distortion in Cftr +/- intercross-derived mice.
X-ray microanalysis of apical fluid in cystic fibrosis airway epithelial cell lines.
X-ray microanalysis of cell elements in normal and cystic fibrosis jejunum: evidence for chloride secretion in villi.
XV-2c/KM19 haplotypes analysis of cystic fibrosis patients from western Mexico.
Yes-associated protein 65 localizes p62(c-Yes) to the apical compartment of airway epithelia by association with EBP50.
Yet another role for the cystic fibrosis transmembrane conductance regulator.
Zinc Deficiency via a Splice Switch in Zinc Importer ZIP2/SLC39A2 Causes Cystic Fibrosis-Associated MUC5AC Hypersecretion in Airway Epithelial Cells.
[(Dihydroindenyl)oxy]alkonic acid inhibits the cystic fibrosis transmembrane conductance regulator.
[3120+1kbdel8.6kb]+[p.N1303K] genotype in an Emirati cystic fibrosis patient: indication of a founder mutation in Palestinian Arabs.
[A case report of cystic fibrosis and review of 16 cases of cystic fibrosis in Chinese patients]
[Abdominal manifestations in cystic fibrosis : Clinical review].
[Aerosol administration of a replication defective recombinant adenovirus expressing normal human cDNA-CFTR in the respiratory tractus in patients with cystic fibrosis]
[AFU and SALF recommendations for the evaluation of male infertility].
[Analysis of the spectra of mutations and polymorphic loci of cystic fibrosis transmembrane conductance regulator in the population of Bashkortostan]
[Antisense oligonucleotides for therapy of cystic fibrosis : Inhibition of sodium absorption mediated by ENaC in nasal epithelial cells.]
[Association of CFTR gene polymorphism with congenital bilateral absence of vas deferens in ethnic Han Chinese patients].
[Benzamil and mucoviscidosis. Primary culture of nasal mucosa as an electrophysiologic in vitro model]
[Butyl-p-hydroxybenzoate stimulates cystic fibrosis transmembrane conductance regulator Cl- transport]
[CF Lung Disease - a German S3 Guideline: Module 2: Diagnostics and Treatment in Chronic Infection with Pseudomonas aeruginosa].
[CFTR and ENaC functions in cystic fibrosis].
[CFTR as cAMP-dependent chloride channels and as cAMP-dependent regulator of sodium channels]
[CFTR gene analyis in 207 patients with cystic fibrosis in southwest France: high frequency of N1303K and 1811+1.6bA>G mutations]
[CFTR gene variations and phenotypes in seven children].
[CFTR protein and molecular mechanisms of pulmonary involvement in cystic fibrosis]
[Clinical and molecular genetic study of cystic fibrosis in the 5th Region of Chile]
[Clinical aspects and genetic specificities of cystic fibrosis in Reunion Island]
[Clinical presentation of lung disease in cystic fibrosis].
[Comparisons of pulmonary and sinonasal lesions in patients with cystic fibrosis. Evaluation using computerized tomography]
[Congenital absence of the vas deferens]
[Correlation between CFTR 5T polymorphisms and the risk of congenital bilateral absence of the vas deferens].
[Correlation between phenotype and genotype in a group of patients with cystic fibrosis]
[Cystic fibrosis and associated complications].
[Cystic fibrosis and other channelopathies].
[Cystic fibrosis being a polyendocrine disease (Review)].
[Cystic fibrosis from the exocrine pancreatic point of view]
[Cystic fibrosis gene mutations in the West of France: clinical application]
[Cystic fibrosis in a woman aged seventy].
[Cystic fibrosis in adults]
[Cystic fibrosis lung disease and its management]
[Cystic fibrosis modifying genes]
[CYSTIC FIBROSIS SURVIVAL TRENDS IN CARMEL MEDICAL CENTER].
[Cystic fibrosis transmembrane conductance regulator (CFTR) gene: mutations and clinical phenotypes]
[Cystic fibrosis: From gene discovery to precision medicine].
[Cystic fibrosis: relationship between genotype and phenotype]
[Cystic fibrosis]
[Detection of deltaF508 deletion causing cystic fibrosis, using quantitative real-time PCR]
[Detection of mutations in the CFTR gene using the PCR-dependent preferential homoduplex formation assay (PCR-PHFA) system]
[Detection of the mutation of all the exons of the CFTR gene in Chinese men with congenital bilateral absence of the vas deferens].
[Diagnosis and management of cystic fibrosis in children]
[DNA analysis and gene diagnosis of cystic fibrosis]
[Dry eye syndrome in children with 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].
[Effects of intratympanic injection of dexamethasone on endolymphatic hydrops and changes in guinea pigs inner ear CFTR expression].
[Effects of monocarboxylic acid derivatives on cardiac ventricular CFTR Cl- channels in guinea pig]
[Epidemiologic study of the cystic fibrosis gene in the Champagne-Ardenne region]
[Establishment and evaluation of methods for determinating cystic fibrosis transmembrane conductance regulator quantitatively].
[Evidence-based treatment of cystic fibrosis].
[Expression of cystic fibrosis transmembrane conductance regulator in human endometrium]
[Expressions of cystic fibrosis transmembrane conductance regulator in epithelial cells of nasal polyps and normal turbinate mucosae]
[Frequency of CFTR gene mutations in idiopathic pancreatitis]
[Frequency of delta F508 mutation in Venezuelan patients with cystic fibrosis]
[From gene to disease; from defective chloride ion transport to cystic fibrosis]
[Functional compartmentation of the endocrine action of cardiac natriuretic peptides]
[Gastrointestinal complications of adult patients with cystic fibrosis]
[Gene therapy in mucoviscidosis]
[Genetic causes of male infertility]
[Genetic mutations as a cause of acute recurrent pancreatitis in children - case report and literature review].
[Genotypes of cystic fibrosis (CF) reported in the world and polymorphisms of cystic fibrosis transmembrane conductance regulator (CFTR) gene in Japanese]
[Identification of mutation in the gene cystic fibrosis transmembrane regulator (CFTR) in Chilean patients with cystic fibrosis]
[Impact of Cystic Fibrosis Transmembrane Conductance Regulator on Malignant? Properties of KRAS Mutant Lung Adenocarcinoma A549 Cells].
[Impact of the CFTR chloride channel on the cytoskeleton of mouse Sertoli cells].
[Inhibitors of intra-cystic secretion in autosomal dominant polycystic kidney disease (ADPKD): a possible way forward].
[Ion transport in nasal and paranasal sinus mucosa in mucoviscidosis and chronic sinusitis]
[Italian Cystic Fibrosis Registry (ICFR). Report 2015-2016].
[Italian Cystic Fibrosis Registry (ICFR). Report 2017-2018].
[Italian Cystic Fibrosis Registry. Report 2011-2014].
[MDR (multidrug resistance) in hepatocarcinoma clinical-therapeutic implications]
[Microbiological diagnosis of bronchopulmonary colonization-infection in cystic fibrosis.]
[Molecular basis of phenotype heterogeneity in 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]
[Molecular diagnosis of cystic fibrosis]
[Molecular genetic analysis of TUB18 and TUB20 intragenic polymorphism and various mutations of the CFTR gene in the Moscow region]
[Molecular genetics principles in cystic fibrosis. An example of genetic illness in pneumology]
[Mucoviscidosis: Background, risk factors, diagnosis strategy, treatment principles]
[Mutation in the cystic fibrosis transmembrane-regulator gene in bilateral congenital ductus deferens aplasia]
[New concepts of pathophysiology and therapy in cystic fibrosis]
[Newborn screening for cystic fibrosis in the Netherlands].
[Pancreatitis in cystic fibrosis: association with genotype and pancreatic status.]
[Pancreatitis--etiology and pathogenesis]
[Pancreatitis-associated gene mutations]
[Patients with cystic fibrosis become adults : Treatment hopes and disappointments].
[Physiopathology of cystic fibrosis lung disease]
[Physiopathology of cystic fibrosis]
[PMP70, the 70-kDa peroxisomal membrane protein: a member of the ATP-binding cassette transporters]
[Polymethoxylated flavonoids activate cystic fibrosis transmembrane conductance regulator chloride channel].
[Possibilities and prospects of the use of DNA analysis in the diagnosis and prevention of inherited disease in the Ukraine]
[Post-translational ligation and function of dual-vector transferred split CFTR gene].
[Post-translational ligation of split CFTR severed before TMD2 and its chloride channel function].
[Problems of gene transfer to human airway in the patients with respiratory diseases]
[Protective effect of Yishen Tongluo Recipe against benzo(a)pyrene-induced sperm DNA methylation changes in male rats].
[Protein repair therapy in cystic fibrosis]
[Quantitative analysis of SMN gene copies in spinal muscular atrophy]
[Real-world effectiveness of ivacaftor in children with cystic fibrosis and the G551D mutation].
[Regulation by vasopressin of NaCl absorption in the renal collecting duct]
[Relationship of Cystic Fibrosis Transmembrane Conductance Regulator Expression with Clinical Features and Prognosis in Patients with Acute Leukemia].
[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 Valparaíso, Chile.]
[Severe male infertility. Genetic investigation and counseling prior to intracytoplasmic sperm injection]
[The activation effect of nobiletin on cystic fibrosis transmembrane conductance regulator chloride channel].
[The cystic fibrosis gene: mutation and the function of CFTR protein]
[Update of genetic evaluation for male infertility].
{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.
A potential strategy for reducing cysts in autosomal dominant polycystic kidney disease with a CFTR corrector.
A regulatory role of polycystin-1 on cystic fibrosis transmembrane conductance regulator plasma membrane expression.
A role for CFTR in human autosomal dominant polycystic kidney disease.
Activating AMP-activated protein kinase (AMPK) slows renal cystogenesis.
Activation of AMP-activated kinase as a strategy for managing autosomal dominant polycystic kidney disease.
Anoctamin 1 induces calcium-activated chloride secretion and proliferation of renal cyst-forming epithelial cells.
Autosomal dominant polycystic kidney disease coexisting with cystic fibrosis.
Basolateral chloride transporters in autosomal dominant polycystic kidney disease.
cAMP regulates cell proliferation and cyst formation in autosomal polycystic kidney disease cells.
CFTR Inhibitors.
Cyst growth in ADPKD is prevented by pharmacological and genetic inhibition of TMEM16A in vivo.
Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth.
Inhibition of histone deacetylase 6 activity reduces cyst growth in polycystic kidney disease.
KCa3.1 potassium channels are critical for cAMP-dependent chloride secretion and cyst growth in autosomal-dominant polycystic kidney disease.
Lipid Peroxidation Drives Renal Cyst Growth In Vitro through Activation of TMEM16A.
Loss of PKD1 and PKD2 share common effects on intracellular Ca2+ signaling.
Mechanism and application of metformin in kidney diseases: An update.
Nanomolar Potency Pyrimido-pyrrolo-quinoxalinedione CFTR Inhibitor Reduces Cyst Size in a Polycystic Kidney Disease Model.
Pharmacological reversal of renal cysts from secretion to absorption suggests a potential therapeutic strategy for managing autosomal dominant polycystic kidney disease.
Role of CFTR in autosomal recessive polycystic kidney disease.
Small-molecule CFTR inhibitors slow cyst growth in polycystic kidney disease.
Steviol Reduces MDCK Cyst Formation and Growth by Inhibiting CFTR Channel Activity and Promoting Proteasome-Mediated CFTR Degradation.
Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease.
The relationship between cell proliferation, Cl- secretion, and renal cyst growth: a study using CFTR inhibitors.
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.
Therapeutic potential of cystic fibrosis transmembrane conductance regulator (CFTR) inhibitors in polycystic kidney disease.
TMEM16A drives renal cyst growth by augmenting Ca2+ signaling in M1 cells.
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.
Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies.
Auto-obliteration of maxillary sinuses through osteoneogenesis in children with cystic fibrosis: A possible new way to reduce morbidity.
Cigarette Smoke-induced Ca2+ Release Leads to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Dysfunction.
Epithelial Na(+) channel inhibitors for the treatment of cystic fibrosis.
Hydrator Therapies for Chronic Bronchitis. Lessons from Cystic Fibrosis.
Immunopathology of Airway Surface Liquid Dehydration Disease.
Inflammation in cystic fibrosis: An update.
Physiological impact of abnormal lipoxin A? production on cystic fibrosis airway epithelium and therapeutic potential.
Reduced NHE3-mediated Na+ absorption increases survival and decreases the incidence of intestinal obstructions in cystic fibrosis mice.
Rheological effects of hypertonic saline and sodium bicarbonate solutions on cystic fibrosis sputum in vitro.
Roflumilast combined with adenosine increases mucosal hydration in human airway epithelial cultures after cigarette smoke exposure.
The cystic fibrosis gene and its product CFTR.
Unplugging Mucus in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Ursodeoxycholic acid inhibits ENaC and Na/K pump activity to restore airway surface liquid height in cystic fibrosis bronchial epithelial cells.
Diabetes Mellitus
Monogenic diabetes mellitus in cystic fibrosis.
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
Isorhamnetin Ameliorates Dry Eye Disease via CFTR Activation in Mice.
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.
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.
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.
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.
A proinflammatory, antiapoptotic phenotype underlies the susceptibility to acute pancreatitis in cystic fibrosis transmembrane regulator (-/-) mice.
Accumulation of ceramide in the trachea and intestine of cystic fibrosis mice causes inflammation and cell death.
CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis.
Characterization of two distinct chloride channels in cultured dog pancreatic duct epithelial cells.
Cystic fibrosis mutation classes in pediatric otitis media - Fickle or faulty?
Heterogeneity of phenotype in two cystic fibrosis patients homozygous for the CFTR exon 11 mutation G551D.
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.
Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report.
Liver cirrhosis and portal hypertension in cystic fibrosis.
Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis.
Pancreatic complications in children with cystic fibrosis.
Pancreatitis in A Patient with Cystic Fibrosis Taking Ivacaftor.
Phenotype of CF and the effects of possible modifier genes.
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.
A Homogeneous Cell-Based Halide-Sensitive Yellow Fluorescence Protein Assay to Identify Modulators of the Cystic Fibrosis Transmembrane Conductance Regulator Ion Channel.
A kinase-regulated mechanism controls CFTR channel gating by disrupting bivalent PDZ domain interactions.
A mutation of the epithelial sodium channel associated with atypical cystic fibrosis increases channel open probability and reduces Na+ self inhibition.
A Novel Cystic Fibrosis Gene Mutation C.4242+1G>C in an Omani Patient: A Case Report.
A novel regulatory role for tissue transglutaminase in epithelial-mesenchymal transition in cystic fibrosis.
A single amino acid substitution in CFTR converts ATP to an inhibitory ligand.
A spatial model of fluid recycling in the airways of the lung.
A SYK/SHC1 pathway regulates the amount of CFTR in the plasma membrane.
Adapting Proteostasis and Autophagy for Controlling the Pathogenesis of Cystic Fibrosis Lung Disease.
Advances In Gene Therapy For Cystic Fibrosis Lung Disease.
Alpha-1 Antitrypsin-A Target for MicroRNA-Based Therapeutic Development for Cystic Fibrosis.
Altering intracellular pH reveals the kinetic basis of intraburst gating in the CFTR Cl(-) channel.
Animal models for cystic fibrosis liver disease (CFLD).
Applicability of different antibodies for immunohistochemical localization of CFTR in sweat glands from healthy controls and from patients with cystic fibrosis.
Aquagenic Wrinkling of the Palm: A Rare Diagnostic Clue of Cystic Fibrosis and the Response to CFTR-Modulating Therapy.
Association of CFTR gene mutation with bronchial asthma.
Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis.
Azithromycin fails to reduce inflammation in cystic fibrosis airway epithelial cells.
Bone disease in cystic fibrosis: new pathogenic insights opening novel therapies.
Calreticulin negatively regulates the cell surface expression of cystic fibrosis transmembrane conductance regulator.
Capsaicin-Loaded Chitosan Nanocapsules for wtCFTR-mRNA Delivery to a Cystic Fibrosis Cell Line.
Cardiovascular complications in cystic fibrosis: A review of the literature.
Caring for Adolescents with Cystic Fibrosis, in Portugal: The Nurse's Role.
Cefoperazone sodium liposomal formulation to mitigate P. aeruginosa biofilm in Cystic fibrosis infection: A QbD approach.
Ceftolozane/tazobactam for pulmonary exacerbation in a 63-year-old cystic fibrosis patient with renal insufficiency and an elevated MIC to Pseudomonas aeruginosa.
CELF proteins regulate CFTR pre-mRNA splicing: essential role of the divergent domain of ETR-3.
Cell-Selective Regulation of CFTR Gene Expression: Relevance to Gene Editing Therapeutics.
CFTR dysfunction in cystic fibrosis and chronic obstructive pulmonary disease.
CFTR Inhibitors.
CFTR Modulators: Impact on Fertility, Pregnancy, and Lactation in Women with Cystic Fibrosis.
CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR.
CFTR regulation by phosphorylation.
CFTR regulation of epithelial sodium channel.
Challenges Facing Airway Epithelial Cell-Based Therapy for Cystic Fibrosis.
Characterization of an intron 12 splice donor mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia.
Chloride Conductance, Nasal Potential Difference and Cystic Fibrosis Pathophysiology.
Cigarette Smoke and CFTR: Implications in the pathogenesis of COPD.
Clinically-approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia.
Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis.
Correction of the CF defect by curcumin: hypes and disappointments.
Corticosteroid use and increased CXCR2 levels on leukocytes are associated with lumacaftor/ivacaftor discontinuation in cystic fibrosis patients homozygous for the F508del CFTR mutation.
Current Status of Genetic Diagnosis Laboratories and Frequency of Genetic Variants Associated with Cystic Fibrosis through a Newborn-Screening Program in Turkey.
Cystic fibrosis and innate immunity: how chloride channel mutations provoke lung disease.
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 transmembrane conductance regulator degradation depends on the lectins Htm1p/EDEM and the Cdc48 protein complex in yeast.
Cystic Fibrosis Transmembrane Conductance Regulator Genotype, Not Circulating Catecholamines, Influences Cardiovascular Function in Patients with Cystic Fibrosis.
Cystic fibrosis transmembrane conductance regulator-emerging regulator of cancer.
Cystic fibrosis, the CFTR, and rectifying Cl- channels.
Cystic Fibrosis: Overview of the Current Development Trends and Innovative Therapeutic Strategies.
Cystic fibrosis: Physiopathology and the latest pharmacological treatments.
Cytokine pattern in cystic fibrosis patients during antibiotic therapy and gene therapy using adenoviral vector.
Dataset of differential lipid raft and global proteomes of SILAC-labeled cystic fibrosis cells upon TNF -? stimulation.
Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition.
Defective CFTR-dependent CREB activation results in impaired spermatogenesis and azoospermia.
Deletion of cftr Leads to an Excessive Neutrophilic Response and Defective Tissue Repair in a Zebrafish Model of Sterile Inflammation.
Development of CFTR Structure.
Diagnostic tools and CFTR functional assays in cystic fibrosis: utility and availability in Switzerland.
Differential function of the two nucleotide binding domains on cystic fibrosis transmembrane conductance regulator.
Differential sensitivity of the cystic fibrosis (CF)-associated mutants G551D and G1349D to potentiators of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel.
Discovery of GLPG2451, a Novel Once Daily Potentiator for the Treatment of Cystic Fibrosis.
Dysfunction of the non-neuronal cholinergic system in the airways and blood cells of patients with cystic fibrosis.
Dysregulated signalling pathways in innate immune cells with cystic fibrosis mutations.
Electrophysiological Approaches for the Study of Ion Channel Function.
Elevated tear fluid levels of MIP-1alpha in patients with cystic fibrosis.
Epithelial mesenchymal transition (EMT): a universal process in lung diseases with implications for cystic fibrosis pathophysiology.
Establishment and characterization of a novel polarized MDCK epithelial cellular model for CFTR studies.
Evolving Mechanistic Views and Emerging Therapeutic Strategies for Cystic Fibrosis-Related Diabetes.
Fas and Fas ligand expression in cystic fibrosis airway epithelium.
Fas Expression in Conjunctival Epithelial Cells of Patients With Cystic Fibrosis.
Folding and rescue of a cystic fibrosis transmembrane conductance regulator trafficking mutant identified using human-murine chimeric proteins.
Functional characterization reveals that zebrafish CFTR prefers to occupy closed channel conformations.
Functional Gene Correction for Cystic Fibrosis in Lung Epithelial Cells Generated from Patient iPSCs.
Gene modifiers of lung disease.
Generation of an induced pluripotent stem cell line (MHHi018-A) from a patient with Cystic Fibrosis carrying p.Asn1303Lys (N1303K) mutation.
Genetics of Cystic Fibrosis: Clinical Implications.
Glibenclamide stimulates fluid secretion in rodent cholangiocytes through a cystic fibrosis transmembrane conductance regulator-independent mechanism.
High-Throughput Screening for Readthrough Modulators of CFTR PTC Mutations.
Human Genetics Society of Australasia position statement: population-based carrier screening for cystic fibrosis.
Identification and membrane localization of electrogenic sodium bicarbonate cotransporters in Calu-3 cells.
Immune response of polarized cystic fibrosis airway epithelial cells infected with Influenza A virus.
Impact of the F508del mutation on ovine CFTR, a Cl(-) channel with enhanced conductance and ATP-dependent gating.
Impaired CFTR-Dependent Amplification of FSH-Stimulated Estrogen Production in Cystic Fibrosis and PCOS.
In vivo evaluation of the safety of adenovirus-mediated transfer of the human cystic fibrosis transmembrane conductance regulator cDNA to the lung.
In-vivo crystals reveal critical features of the interaction between CFTR and the PDZ2 domain of Na+/H+ exchange cofactor NHERF1.
Incidence, Prevalence, Etiology, and Prognosis of First-Time Chronic Pancreatitis in Young Patients: A Nationwide Cohort Study.
Increased arylsulfatase B activity in cystic fibrosis cells following correction of CFTR.
Increased Folding and Channel Activity of a Rare Cystic Fibrosis Mutant with CFTR Modulators.
Insight into cystic fibrosis by structural modelling of CFTR first nucleotide binding fold (NBF1).
Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis.
Ion Channel Modulators in Cystic Fibrosis.
Iron chelation as novel treatment for lung inflammation in cystic fibrosis.
Matrine modulates HSC70 levels and rescues ?F508-CFTR.
Mechanisms of protein misfolding in conformational lung diseases.
Mechanosensitive gating of CFTR.
Mechanosensitivity of wild-type and G551D cystic fibrosis transmembrane conductance regulator (CFTR) controls regulatory volume decrease in simple epithelia.
Metabolic Reprograming of Cystic Fibrosis Macrophages via the IRE1? Arm of the Unfolded Protein Response Results in Exacerbated Inflammation.
Methylomic correlates of autophagy activity in cystic fibrosis.
Mitochondrial oxidative stress in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice.
Mitochondrial Stress Responses and "Mito-Inflammation" in Cystic Fibrosis.
Model of mucociliary clearance in cystic fibrosis lungs.
Modeling the Conformational Changes Underlying Channel Opening in CFTR.
Molecular Dynamics Flexible Fitting Simulations Identify New Models of the Closed State of the Cystic Fibrosis Transmembrane Conductance Regulator Protein.
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin.
Nanoparticles that deliver triplex-forming peptide nucleic acid molecules correct F508del CFTR in airway epithelium.
Non-specific activation of the epithelial sodium channel by the CFTR chloride channel.
Nonequilibrium gating of CFTR on an equilibrium theme.
Novel frameshift variant of the CFTR gene: S511Lfs*2 from phenotype to molecular predictions.
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.
Nutritional effects on host response to lung infections with mucoid Pseudomonas aeruginosa in mice.
On the discovery and development of CFTR chloride channel activators.
On the interactions between nucleotide binding domains and membrane spanning domains in cystic fibrosis transmembrane regulator: A molecular dynamic study.
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.
Pharmacological analysis of CFTR variants of cystic fibrosis using stem cell-derived organoids.
Pharmacological approaches to correcting the ion transport defect in cystic fibrosis.
Pharmacological treatment of the basic defect in 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.
Pitfalls in the immunohistochemical localization of the cystic fibrosis transmembrane conductance regulator in paraffin embedded sweat glands.
Potentiation of the cystic fibrosis transmembrane conductance regulator by VX-770 involves stabilization of the pre-hydrolytic, O1 state.
Pseudomonas aeruginosa triggers CFTR-mediated airway surface liquid secretion in swine trachea.
Pulmonary bacterial pathogens in cystic fibrosis patients and antibiotic therapy: a tool for the health workers.
RACK1 interacts with filamin-A to regulate plasma membrane levels of the cystic fibrosis transmembrane conductance regulator.
Recent Progress in the Discovery and Development of Small-Molecule Modulators of CFTR.
Relating the Disease Mutation Spectrum to the Evolution of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Resveratrol rescues cAMP-dependent anionic transport in the cystic fibrosis pancreatic cell line CFPAC1.
Role of individual R domain phosphorylation sites in CFTR regulation by protein kinase A.
Role of the amiloride-sensitive epithelial Na+ channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease.
Selectively targeting key inflammatory pathways in cystic fibrosis.
Short communication: novel truncating mutations in the CFTR gene causing a severe form of cystic fibrosis in Italian patients.
Sleep-Related Rhythmic Movement Disorder in Triplets: Evidence for Genetic Predisposition?
Small Molecule Anion Carriers Correct Abnormal Airway Surface Liquid Properties in Cystic Fibrosis Airway Epithelia.
Specific resistance to Pseudomonas aeruginosa infection in zebrafish is mediated by the cystic fibrosis transmembrane conductance regulator.
Structural models of CFTR-AMPK and CFTR-PKA interactions: R-domain flexibility is a key factor in CFTR regulation.
Subepithelial fibrosis and degradation of the bronchial extracellular matrix in cystic fibrosis.
Synthesis and biological evaluation of thiazole derivatives on basic defects underlying cystic fibrosis.
Synthesis and properties of molecular probes for the rescue site on mutant cystic fibrosis transmembrane conductance regulator.
Testicular Cancer in a Lung Transplant Patient With Cystic Fibrosis: A Case Report.
The Application of Bicarbonate Recovers the Chemical-Physical Properties of Airway Surface Liquid in Cystic Fibrosis Epithelia Models.
The bidirectional relationship between CFTR and lipids.
The block of CFTR by scorpion venom is state-dependent.
The cystic fibrosis mutation G1349D within the signature motif LSHGH of NBD2 abolishes the activation of CFTR chloride channels by genistein.
The Potential Causes of Cystic Fibrosis-Related Diabetes.
The Role of Specialized Pro-Resolving Mediators in Cystic Fibrosis Airways Disease.
Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion.
Transmembrane domain of cystic fibrosis transmembrane conductance regulator: design, characterization, and secondary structure of synthetic peptides m1-m6.
Triplet CFTR modulators: future prospects for treatment of cystic fibrosis.
Unplugging Mucus in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Unravelling the Regions of Mutant F508del-CFTR More Susceptible to the Action of Four Cystic Fibrosis Correctors.
Unveiling the degradative route of the V247M ?-sarcoglycan mutant responsible for LGMD-2D.
Uptake of fluorescent dyes associated with the functional expression of the cystic fibrosis transmembrane conductance regulator in epithelial cells.
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.
Widespread airway distribution and short-term phenotypic correction of cystic fibrosis pigs following aerosol delivery of piggyBac/adenovirus.
Widespread and efficient marker gene expression in the airway epithelia of fetal sheep after minimally invasive tracheal application of recombinant adenovirus in utero.
Worldwide genetic analysis of the CFTR region.
[Antisense oligonucleotides for therapy of cystic fibrosis : Inhibition of sodium absorption mediated by ENaC in nasal epithelial cells.]
[Cystic fibrosis: From gene discovery to precision medicine].
[Microbiological diagnosis of bronchopulmonary colonization-infection in cystic fibrosis.]
[Physiopathology of cystic fibrosis lung disease]
[Post-translational ligation and function of dual-vector transferred split CFTR gene].
[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.
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.
Gonadal Dysgenesis, Mixed
Genetic diagnostics of male infertility in clinical practice.
Heart Failure
Loss of the normal epicardial to endocardial gradient of cftr mRNA expression in the hypertrophied rabbit left ventricle.
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.
Hepatitis B
Origin and utility of the reverse dot-blot.
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.
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.
In vivo investigation of acidified pepsin exposure to porcine vocal fold epithelia.
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.
Hypersensitivity
Beta-lactam allergy in adults with cystic fibrosis.
Hypertension
Autophagy and inflammation in chronic respiratory disease.
[Regulation by vasopressin of NaCl absorption in the renal collecting duct]
Hypertension, Portal
Hepatic manifestations of cystic fibrosis.
Obliterative Portal Venopathy Without Cirrhosis Is Prevalent in Pediatric Cystic Fibrosis Liver Disease With Portal Hypertension.
Hypertension, Pulmonary
Aerosolized Medications for Gene and Peptide Therapy.
Autophagy and inflammation in chronic respiratory disease.
Hypogonadism
[AFU and SALF recommendations for the evaluation of male infertility].
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.
Expression of CFTR in human and bovine thyroid epithelium.
Idiopathic Pulmonary Fibrosis
Autophagy and inflammation in chronic respiratory disease.
Infections
16alpha-Bromoepiandrosterone (HE2000) limits non-productive inflammation and stimulates immunity in lungs.
A Link Between a Common Mutation in CFTR and Impaired Innate and Adaptive Viral Defense.
A spatial model of fluid recycling in the airways of the lung.
AAV exploits subcellular stress associated with inflammation, endoplasmic reticulum expansion, and misfolded proteins in models of cystic fibrosis.
Abnormally up-regulated cystic fibrosis transmembrane conductance regulator expression and uterine fluid accumulation contribute to Chlamydia trachomatis-induced female infertility.
Absence of the cystic fibrosis transmembrane regulator (Cftr) from myeloid-derived cells slows resolution of inflammation and infection.
Accumulation of ceramide in the trachea and intestine of cystic fibrosis mice causes inflammation and cell death.
Acute pancreatitis in association with Campylobacter jejuni-associated diarrhea in a 15-year-old with CFTR mutations: is there a link?
Aerosolized Bovine Lactoferrin Counteracts Infection, Inflammation and Iron Dysbalance in A Cystic Fibrosis Mouse Model of Pseudomonas aeruginosa Chronic Lung Infection.
Airway epithelial control of Pseudomonas aeruginosa infection in cystic fibrosis.
Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies.
Airway mucus in cystic fibrosis.
Airway surface liquid composition in mice.
Altered respiratory epithelial cell cytokine production in cystic fibrosis.
Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis.
Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator.
Biomarkers for cystic fibrosis drug development.
Biomarkers in Paediatric Cystic Fibrosis Lung Disease.
Burkholderia cenocepacia O polysaccharide chain contributes to caspase-1-dependent IL-1beta production in macrophages.
Cardiovascular System Involvement in Cystic Fibrosis.
Cefoperazone sodium liposomal formulation to mitigate P. aeruginosa biofilm in Cystic fibrosis infection: A QbD approach.
Ceftolozane/tazobactam for pulmonary exacerbation in a 63-year-old cystic fibrosis patient with renal insufficiency and an elevated MIC to Pseudomonas aeruginosa.
Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis.
CFTR mutations and host susceptibility to Pseudomonas aeruginosa lung infection.
Characterization of CFTR expression in a human pulmonary mucoepidermoid carcinoma cell line, NCI-H292 cells.
Clusters of Cl- channels in CFTR-expressing Sf9 cells switch spontaneously between slow and fast gating modes.
Common CFTR gene variants influence body composition and survival in rural Ghana.
Concurrent amyotrophic lateral sclerosis and cystic fibrosis supports common pathways of pathogenesis.
Cystic fibrosis mutation classes in pediatric otitis media - Fickle or faulty?
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 transmembrane conductance regulator is an epithelial cell receptor for clearance of Pseudomonas aeruginosa from the lung.
Cystic Fibrosis Transmembrane Conductance Regulator Reduces Microtubule-Dependent Campylobacter jejuni Invasion.
Cystic fibrosis year in review 2018, part 1.
Cystic fibrosis year in review 2019: Section 3 multisystem-based care and research.
Cystic fibrosis: pathogenesis and future treatment strategies.
Disruption of CFTR-dependent lipid rafts reduces bacterial levels and corneal disease in a murine model of Pseudomonas aeruginosa keratitis.
Effects of rhinovirus infection on the expression and function of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel in human nasal mucosa.
Enhancing Cystic Fibrosis Immune Regulation.
Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa.
Gene therapy in a xenograft model of cystic fibrosis lung corrects chloride transport more effectively than the sodium defect.
Genomewide association analysis of respiratory syncytial virus infection in mice.
Helicobacter pylori infection downregulates duodenal CFTR and SLC26A6 expressions through TGF? signaling pathway.
How mutant CFTR may contribute to Pseudomonas aeruginosa infection in cystic fibrosis.
Human Genetic Variation Influences Enteric Fever Progression.
Immune response of polarized cystic fibrosis airway epithelial cells infected with Influenza A virus.
In vitro assessment of variables affecting the efficiency and efficacy of adenovirus-mediated gene transfer to cystic fibrosis airway epithelia.
Increased susceptibility of cftr-/- mice to LPS-induced lung remodeling.
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.
Inflammatory epithelial cytokines after in vitro respiratory syncytial viral infection are associated with reduced lung function.
Influence of cystic fibrosis transmembrane conductance regulator on gene expression in response to Pseudomonas aeruginosa infection of human bronchial epithelial cells.
Influenza virus M2 targets cystic fibrosis transmembrane conductance regulator for lysosomal degradation during viral infection.
Innovating cystic fibrosis clinical trial designs in an era of successful standard of care therapies.
Involvement of cystic fibrosis transmembrane conductance regulator (CFTR) in the pathogenesis of hydrosalpinx induced by Chlamydia trachomatis infection.
Involvement of cystic fibrosis transmembrane conductance regulator in infection-induced edema.
Low sphingosine-1-phosphate impairs lung dendritic cells in cystic fibrosis.
Mid-trimester hyperechogenic bowel in a fetus of Japanese origin carrying a new mutation of CFTR gene (L548Q).
Modification of development by the CFTR gene in utero.
Mucociliary clearance in cystic fibrosis knockout mice infected with Pseudomonas aeruginosa.
Novel AAV-mediated gene delivery system corrects CFTR function in pigs.
Nutritional effects on host response to lung infections with mucoid Pseudomonas aeruginosa in mice.
Optimal Complement-Mediated Phagocytosis of Pseudomonas aeruginosa by Monocytes Is Cystic Fibrosis Transmembrane Conductance Regulator-Dependent.
Pathogen acquisition in patients with cystic fibrosis receiving ivacaftor or lumacaftor/ivacaftor.
Pathophysiology and management of pulmonary infections in cystic fibrosis.
Physiological impact of abnormal lipoxin A? production on cystic fibrosis airway epithelium and therapeutic potential.
Progression of early structural lung disease in young children with cystic fibrosis assessed using CT.
Prospective analysis of cystic fibrosis transmembrane regulator mutations in adults with bronchiectasis or pulmonary nontuberculous mycobacterial infection.
Pseudomonas aeruginosa alginate promotes Burkholderia cenocepacia persistence in cystic fibrosis transmembrane conductance regulator knockout mice.
Pulmonary nontuberculous mycobacterial disease: prospective study of a distinct preexisting syndrome.
Reduced GM1 Ganglioside in CFTR-Deficient Human Airway Cells Results in Decreased ss1-integrin Signaling and Delayed Wound Repair.
Regulation of Pseudomonas aeruginosa internalization after contact lens wear in vivo and in serum-free culture by ocular surface cells.
Regulation of the Coxsackie and adenovirus receptor expression is dependent on cystic fibrosis transmembrane regulator in airway epithelial cells.
Respiratory syncytial virus infection disrupts monolayer integrity and function in cystic fibrosis airway cells.
Respiratory System Disease.
Response to Acute Lung Infection with Mucoid Pseudomonas aeruginosa in Cystic Fibrosis Mice.
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.
Rhesus ?-defensin-1 (RTD-1) exhibits in vitro and in vivo activity against cystic fibrosis strains of Pseudomonas aeruginosa.
Role for cystic fibrosis transmembrane conductance regulator protein in a glutathione response to bronchopulmonary pseudomonas infection.
Role of CD95 in pulmonary inflammation and infection in cystic fibrosis.
Role of CFTR expressed by neutrophils in modulating acute lung inflammation and injury in mice.
Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections.
Role of the cystic fibrosis transmembrane conductance regulator in internalization of Pseudomonas aeruginosa by polarized respiratory epithelial cells.
Specific resistance to Pseudomonas aeruginosa infection in zebrafish is mediated by the cystic fibrosis transmembrane conductance regulator.
Src signaling links mediators of inflammation to Cx43 gap junction channels in primary and transformed CFTR-expressing airway cells.
The Sweat Metabolome of Screen-Positive Cystic Fibrosis Infants: Revealing Mechanisms beyond Impaired Chloride Transport.
Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.
Ursodeoxycholic acid inhibits ENaC and Na/K pump activity to restore airway surface liquid height in cystic fibrosis bronchial epithelial cells.
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.
Clinical genetic testing for male factor infertility: current applications and future directions.
Cystic fibrosis transmembrane conductance regulator mutations in azoospermic and oligospermic men and their partners.
Differential cellular expression of cystic fibrosis transmembrane regulator in human reproductive tissues. Clues for the infertility in patients with cystic fibrosis.
Early activation of sperm by HCO(3)(-) is regulated hormonally in the murine uterus.
Endocervical metaplasia of the endometrium in a patient with cystic fibrosis: a case report.
Female cystic fibrosis mutation carriers and assisted reproductive technology: does carrier status affect reproductive outcomes?
Gender-sensitive association of CFTR gene mutations and 5T allele emerging from a large survey on infertility.
Involvement of CFTR in oviductal HCO3- secretion and its effect on soluble adenylate cyclase-dependent early embryo development.
Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report.
Loss of SLC9A3 decrease CFTR protein and causes obstructed azoospermia in mice.
Screening for mutations in the cystic fibrosis transmembrane regulator gene in an infertility clinic.
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.
A novel mutation (-195C>A) in the promoter region of CFTR gene is associated with Chinese Congenital Bilateral Absence of Vas Deferens (CBAVD).
Carbamazepine-induced sperm disorders can be associated with the altered expressions of testicular KCNJ11/miR-let-7a and spermatozoal CFTR/miR-27a.
CFTR gene mutations and male infertility.
Clinical genetic testing for male factor infertility: current applications and future directions.
Congenital absence of vas deferens and cystic fibrosis.
Cystic fibrosis gene mutations and polymorphisms in Saudi men with infertility.
Genetic cystic fibrosis transmembrane regulator 4016insT D1152H compound heterozygosity and male infertility: an Italian case report.
Genetic diagnostics of male infertility in clinical practice.
Genetic markers of male infertility: Y chromosome microdeletions and cystic fibrosis transmembrane conductance gene mutations.
Impact of Cystic Fibrosis Transmembrane Regulator (CFTR) gene mutations on male infertility.
Is intracytoplasmic sperm injection itself an indication to perform preimplantation genetic diagnosis (PGD)? About PGD, invasive prenatal diagnosis and genetic sonography.
Linkage disequilibrium between the M470V variant and the IVS8 polyT alleles of the CFTR gene in CBAVD.
Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis.
Molecular analysis of mutations and polymorphisms in the CFTR gene in male infertility.
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.
Molecular screening of the CFTR gene in men with anomalies of the vas deferens: identification of three novel mutations.
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.
Mutations in CFTR gene and clinical correlation in Argentine patients with congenital bilateral absence of the vas deferens.
Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens.
Regulation of male fertility by CFTR and implications in male infertility.
Screening of ?F508 mutation and IVS8-poly T polymorphism in CFTR gene in Tunisian infertile men without CBAVD.
Study of mutant and polyvariant mutant CFTR genes in patients with congenital absence of the vas deferens.
The CFTR Met 470 allele is associated with lower birth rates in fertile men from a population isolate.
The cystic fibrosis transmembrane regulator gene and male infertility.
[Genetic causes of male infertility]
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.
Heterozygosity for the F508del mutation in the cystic fibrosis transmembrane conductance regulator anion channel attenuates influenza severity.
Influenza virus M2 targets cystic fibrosis transmembrane conductance regulator for lysosomal degradation during viral infection.
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
Intestinal bicarbonate secretion in cystic fibrosis mice.
Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota.
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.
Clinical and genetic risk factors for cystic fibrosis-related liver disease.
Distal intestinal obstruction syndrome in adults with cystic fibrosis.
Growth deficits in cystic fibrosis mice begin in utero prior to IGF-1 reduction.
Liver cirrhosis and portal hypertension in cystic fibrosis.
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.
Kallmann Syndrome
Genetic diagnostics of male infertility in clinical practice.
Kidney Calculi
ClC and CFTR chloride channel gating.
Klinefelter Syndrome
Genetic diagnostics of male infertility in clinical practice.
Transmission of male infertility and intracytoplasmic sperm injection (mini-review).
Lesch-Nyhan Syndrome
PCR from single cells for preimplantation diagnosis.
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.
Unconventional secretion of transmembrane proteins.
[Relationship of Cystic Fibrosis Transmembrane Conductance Regulator Expression with Clinical Features and Prognosis in Patients with Acute Leukemia].
Liver Diseases
Animal models for cystic fibrosis liver disease (CFLD).
CFTR expression and mucin secretion in cultured mouse gallbladder epithelial cells.
Elevated hepatic SULT1E1 activity in mouse models of cystic fibrosis alters the regulation of estrogen responsive proteins.
Glibenclamide stimulates fluid secretion in rodent cholangiocytes through a cystic fibrosis transmembrane conductance regulator-independent mechanism.
Hepatic manifestations of cystic fibrosis.
Hepatobiliary disease in patients with cystic fibrosis.
Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report.
Liver disease in cystic fibrosis.
Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR.
Multi-dimensional clinical phenotyping of a national cohort of adult cystic fibrosis patients.
Obliterative Portal Venopathy Without Cirrhosis Is Prevalent in Pediatric Cystic Fibrosis Liver Disease With Portal Hypertension.
Pathobiology of inherited biliary diseases: a roadmap to understand acquired 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.
A placebo-controlled study of liposome-mediated gene transfer to the nasal epithelium of patients with cystic fibrosis.
Aspergillus-induced superoxide production by cystic fibrosis phagocytes is associated with disease severity.
Association of cystic fibrosis genetic modifiers with congenital bilateral absence of the vas deferens.
Associations between peak oxygen uptake, lung function, and bronchiectasis in children with cystic fibrosis in the era of CFTR modulators.
Autophagy Augmentation to Alleviate Immune Response Dysfunction, and Resolve Respiratory and COVID-19 Exacerbations.
Biomarkers in Paediatric Cystic Fibrosis Lung Disease.
CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells.
CFTR gene mutations in adults with disseminated bronchiectasis.
CFTR gene mutations--including three novel nucleotide substitutions--and haplotype background in patients with asthma, disseminated bronchiectasis and chronic obstructive pulmonary disease.
CFTR transgene expression in primary DeltaF508 epithelial cell cultures from human nasal polyps following gene transfer with cationic phosphonolipids.
CFTR-deficient pigs display alterations of bone microarchitecture and composition at birth.
CFTR-function and ventilation inhomogeneity in individuals with cystic fibrosis.
CFTR: cystic fibrosis and beyond.
Cigarette Smoke-Induced Acquired Dysfunction of Cystic Fibrosis Transmembrane Conductance Regulator in the Pathogenesis of Chronic Obstructive Pulmonary Disease.
ClC-5: ontogeny of an alternative chloride channel in respiratory epithelia.
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
Current concepts of immune dysregulation in cystic fibrosis.
Cystic Fibrosis Related Diabetes - An Update.
Cystic fibrosis swine fail to secrete airway surface liquid in response to inhalation of pathogens.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Asians with chronic pulmonary disease: a pilot study.
Cystic Fibrosis Transmembrane Conductance Regulator-independent Phagosomal Acidification in Macrophages.
Cystic Fibrosis Transmembrane Conductance Regulator. Implications in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Cystic fibrosis with three mutations in the cystic fibrosis transmembrane conductance regulator gene.
Cystic fibrosis year in review 2019: Section 3 multisystem-based care and research.
Cystic fibrosis.
Cystic fibrosis: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases.
DEFECTIVE ORGANELLAR ACIDIFICATION AS A CAUSE OF CYSTIC FIBROSIS LUNG DISEASE - RE-EXAMINATION OF A RECURRING HYPOTHESIS.
Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function.
Designing Clinical Trials for Anti-Inflammatory Therapies in Cystic Fibrosis.
Detection of modifier loci influencing the lung phenotype of cystic fibrosis knockout mice.
Discovery of Icenticaftor (QBW251), a Cystic Fibrosis Transmembrane Conductance Regulator Potentiator with Clinical Efficacy in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Discovery of Multitarget Agents Active as Broad-Spectrum Antivirals and Correctors of Cystic Fibrosis Transmembrane Conductance Regulator for Associated Pulmonary Diseases.
EDNRA variants associate with smooth muscle mRNA levels, cell proliferation rates, and cystic fibrosis pulmonary disease severity.
Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host defense.
Evidence for safety and efficacy of DOTAP cationic liposome mediated CFTR gene transfer to the nasal epithelium of patients with cystic fibrosis.
Expression of alpha v beta 5 integrin is necessary for efficient adenovirus-mediated gene transfer in the human airway.
Expression of cystic fibrosis transmembrane conductance regulator in the human distal lung.
Expression of S100A8 correlates with inflammatory lung disease in congenic mice deficient of the cystic fibrosis transmembrane conductance regulator.
Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis.
Genetic modifiers of lung disease in cystic fibrosis.
Genotype-phenotype relationships in a cohort of adult cystic fibrosis patients.
Harnessing Neutrophil Survival Mechanisms during Chronic Infection by Pseudomonas aeruginosa: Novel Therapeutic Targets to Dampen Inflammation in Cystic Fibrosis.
How do cystic fibrosis transmembrane conductance regulator mutations produce lung disease?
Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance.
Hypertonic saline increases lung epithelial lining fluid glutathione and thiocyanate: two protective CFTR-dependent thiols against oxidative injury.
In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in 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.
Inefficient gene transfer by adenovirus vector to cystic fibrosis airway epithelia of mice and humans.
Inhaled interventions in cystic fibrosis: mucoactive and antibiotic therapies.
Inhibition of histone-deacetylase activity rescues inflammatory cystic fibrosis lung disease by modulating innate and adaptive immune responses.
Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis.
Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report.
Left ventricular and aortic dysfunction in cystic fibrosis mice.
Loss of Cftr function exacerbates the phenotype of Na+ hyperabsorption in murine airways.
Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis.
Modulation of cystic fibrosis lung disease by variants in interleukin-8.
New concepts of the pathogenesis of cystic fibrosis lung disease.
Nicotine Induces Progressive Properties of Lung Adenocarcinoma A549 Cells by Inhibiting Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Expression and Plasma Membrane Localization.
Nitric oxide synthase 1 as a potential modifier gene of decline in lung function in patients with cystic fibrosis.
Pathophysiology and management of pulmonary infections in cystic fibrosis.
Phenotype of CF and the effects of possible modifier genes.
Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia.
Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTATM.
Predictors of deterioration of lung function in cystic fibrosis.
Proteases and antiproteases in cystic fibrosis: pathogenetic considerations and therapeutic strategies.
Quantification of Phenotypic Variability of Lung Disease in Children with 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.
Respiratory epithelial gene expression in patients with mild and severe cystic fibrosis lung disease.
Respiratory syncytial virus infection in a murine model of 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.
Safety of aerosolized INS 365 in patients with mild to moderate cystic fibrosis: results of a phase I multi-center study.
Sphingolipids and plasma membrane hydrolases in human primary bronchial cells during differentiation and their altered patterns in cystic fibrosis.
SPX-101 Is a Novel Epithelial Sodium Channel-targeted Therapeutic for Cystic Fibrosis That Restores Mucus Transport.
Strain-dependent pulmonary gene expression profiles of a cystic fibrosis mouse model.
Susceptibility to nontuberculous mycobacterial lung disease.
The CFTR Mutation c.3453G > C (D1152H) Confers an Anion Selectivity Defect in Primary Airway Tissue that Can Be Rescued by Ivacaftor.
The effect of chloride concentration on human neutrophil functions: potential relevance to cystic fibrosis.
The need for vigilance: the case of a false-negative newborn screen for cystic fibrosis.
The yin and yang of cystic fibrosis transmembrane conductance regulator function: implications for chronic lung disease.
Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.
Unplugging Mucus in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Variants in the glutamate-cysteine-ligase gene are associated with cystic fibrosis lung disease.
Vector-specific complementation profiles of two independent primary defects in cystic fibrosis airways.
Lung Diseases, Interstitial
Autophagy and inflammation in chronic respiratory disease.
Lung Diseases, Obstructive
Cystic Fibrosis Transmembrane Conductance Regulator. Implications in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
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.
Critical modifier role of membrane-cystic fibrosis transmembrane conductance regulator-dependent ceramide signaling in lung injury and emphysema.
Ursodeoxycholic acid inhibits ENaC and Na/K pump activity to restore airway surface liquid height in cystic fibrosis bronchial epithelial cells.
Lung Neoplasms
Aerosolized Medications for Gene and Peptide Therapy.
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
Cystic fibrosis transmembrane conductance regulator gene mutation and lung cancer risk.
Nicotine Induces Progressive Properties of Lung Adenocarcinoma A549 Cells by Inhibiting Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Expression and Plasma Membrane Localization.
The cystic fibrosis transmembrane conductance regulator as a biomarker in non-small cell lung cancer.
[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.
Cardiovascular System Involvement in Cystic Fibrosis.
Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth.
The need for vigilance: the case of a false-negative newborn screen for cystic fibrosis.
Meconium Ileus
Clinical and genetic risk factors for cystic fibrosis-related liver disease.
Clinically Applicable Procedure for Gene Delivery to Fetal Gut by Ultrasound-Guided Gastric Injection: Toward Prenatal Prevention of Early-Onset Intestinal Diseases.
Clinically applicable procedure for gene delivery to fetal gut by ultrasound-guided gastric injection: toward prenatal prevention of early-onset intestinal diseases.
Liver cirrhosis and portal hypertension in cystic fibrosis.
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.
Clinical outcomes in infants with cystic fibrosis transmembrane conductance regulator (CFTR) related metabolic syndrome.
Cystic fibrosis infant care challenges in diagnosis and management in the era of newborn screening.
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 Atrophy, Spinal
Small-Molecule Screening for Genetic Diseases.
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.
Heterozygous Cystic Fibrosis Transmembrane Regulator Gene Missense Variants Are Associated With Worse Cardiac Function in Patients With Duchenne Muscular Dystrophy.
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.
Myotonia
ClC and CFTR chloride channel gating.
Nasal Polyps
Localization of cystic fibrosis transmembrane conductance regulator in epithelial cells of nasal polyps and postoperative polypoid mucosae.
The cystic fibrosis conductance regulator gene exon sequence is normal in a patient with edematous eosinophilic 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.
A novel transporter, Pfcrt, confers antimalarial drug resistance.
ABC gene expression profiles have clinical importance and possibly form a new hallmark of cancer.
Adenovirus?mediated overexpression of cystic fibrosis transmembrane conductance regulator enhances invasiveness and motility of serous ovarian cancer cells.
Association of cystic fibrosis transmembrane conductance regulator (CFTR) mutation/variant/haplotype and tumor necrosis factor (TNF) promoter polymorphism in hyperlipidemic pancreatitis.
Autophagy and inflammation in chronic respiratory disease.
CFTR deltaF508 carrier status, risk of breast cancer before the age of 40 and histological grading in a population-based case-control study.
CFTR Functions as a Tumor Suppressor and Is Regulated by DNA Methylation in Colorectal Cancer.
CFTR is a potential marker for nasopharyngeal carcinoma prognosis and metastasis.
Cl- and K+ transport in human biliary cell lines.
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 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 Transmembrane Conductance Regulator Attaches Tumor Suppressor PTEN to the Membrane and Promotes Anti Pseudomonas aeruginosa Immunity.
Cystic fibrosis transmembrane conductance regulator modulates neurosecretory function in pulmonary neuroendocrine cell-related tumor cell line models.
Cytokine-stimulated nitric oxide production inhibits adenylyl cyclase and cAMP-dependent secretion in cholangiocytes.
Downregulation of CFTR promotes epithelial-to-mesenchymal transition and is associated with poor prognosis of breast cancer.
Drugs and their molecular targets: an updated overview.
Effects and interaction of dietary electrolyte balance and citric acid on the intestinal function of weaned piglets.
Expression of voltage-gated potassium channels in human and mouse colonic carcinoma.
Fluorescence energy transfer detection as a homogeneous DNA diagnostic method.
Gene expression profiling of ATP-binding cassette (ABC) transporters as a predictor of the pathologic response to neoadjuvant chemotherapy in breast cancer patients.
Genetics of chronic obstructive pulmonary disease, beyond a1-antitrypsin deficiency.
High level of CFTR expression is associated with tumor aggression and knockdown of CFTR suppresses proliferation of ovarian cancer in vitro and in vivo.
In vivo investigation of acidified pepsin exposure to porcine vocal fold epithelia.
Investigating hypothetical products from noncoding frames (HyPNoFs).
Knockdown of CFTR enhances sensitivity of prostate cancer cells to cisplatin via inhibition of autophagy.
Methylation profile of the promoter CpG islands of 14 "drug-resistance" genes in hepatocellular carcinoma.
NF-kappa B mediates up-regulation of CFTR gene expression in Calu-3 cells by interleukin-1beta.
Nicotine Induces Progressive Properties of Lung Adenocarcinoma A549 Cells by Inhibiting Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Expression and Plasma Membrane Localization.
Overexpression of cystic fibrosis transmembrane conductance regulator (CFTR) is associated with human cervical cancer malignancy, progression and prognosis.
Pancreatic intraepithelial neoplasia is associated with chronic pancreatitis due to serine protease inhibitor kazal type 1 and cystic fibrosis transmembrane conductance regulator mutations.
Predictive value of cystic fibrosis transmembrane conductance regulator (CFTR) in the diagnosis of gastric cancer.
Role of ion channels in gastrointestinal cancer.
Safety of local delivery of low- and intermediate-dose adenovirus gene transfer vectors to individuals with a spectrum of morbid conditions.
Small-Molecule Screening for Genetic Diseases.
The Enigmatic Gut in Cystic Fibrosis: Linking Inflammation, Dysbiosis, and the Increased Risk of Malignancy.
The new frontier: gene and oligonucleotide therapy.
Therapeutically Targeting Tumor Necrosis Factor-?/Sphingosine-1-Phosphate Signaling Corrects Myogenic Reactivity in Subarachnoid Hemorrhage.
TRIM44 links the UPS to SQSTM1/p62-dependent aggrephagy and removing misfolded proteins.
Tumor necrosis factor modulation of expression of the cystic fibrosis transmembrane conductance regulator gene.
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.
Upregulation of CFTR in patients with endometriosis and its involvement in NF?B-uPAR dependent cell migration.
Varied clinical significance of ATP-binding cassette C sub-family members for lung adenocarcinoma.
[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
Genetic evaluation of male infertility.
Mutation frequency of cystic fibrosis transmembrane regulator is not increased in oligozoospermic male candidates for intracytoplasmic sperm injection.
Transmission of male infertility and intracytoplasmic sperm injection (mini-review).
[Update of genetic evaluation for male infertility].
Opioid-Induced Constipation
Mashiningan Improves Opioid-Induced Constipation in Rats by Activating Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel.
Phenylquinoxalinone CFTR activator as potential prosecretory therapy for constipation.
Osteoporosis
Fenretinide prevents the development of osteoporosis in Cftr-KO mice.
Osteoporosis, Postmenopausal
Aerosolized Medications for Gene and Peptide Therapy.
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.
Cystic fibrosis transmembrane regulator mutations and pancreatic disease: closing the gap between genotype and phenotype.
The porcine lung as a potential model for cystic fibrosis.
Pancreatic Neoplasms
CFTR, SPINK1, PRSS1, and CTRC Mutations Are Not Associated With Pancreatic Cancer in German Patients.
Chronic pancreatitis.
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.
A Patient With Pancreas Divisum, Recurrent Acute Pancreatitis, and Homozygosity for the Cystic Fibrosis Transmembrane Regulator-Associated Protein 5T Allele.
A proinflammatory, antiapoptotic phenotype underlies the susceptibility to acute pancreatitis in cystic fibrosis transmembrane regulator (-/-) mice.
A rare mutation in cystic fibrosis transmembrane conductance regulator gene in a recurrent pancreatitis patient without respiratory symptoms.
Acute pancreatitis in association with Campylobacter jejuni-associated diarrhea in a 15-year-old with CFTR mutations: is there a link?
Acute Recurrent and Chronic Pancreatitis as Initial Manifestations of Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders.
Alcohol disrupts levels and function of the cystic fibrosis transmembrane conductance regulator to promote development of pancreatitis.
Analysis of the entire coding region of the cystic fibrosis transmembrane regulator gene in idiopathic pancreatitis.
Association of cystic fibrosis transmembrane conductance regulator (CFTR) mutation/variant/haplotype and tumor necrosis factor (TNF) promoter polymorphism in hyperlipidemic pancreatitis.
Association of pancreas divisum and recurrent acute pancreatitis with the IVS8-5T-12TG allele of the CFTR gene and CFTR dysfunction.
CFTR gene mutations in pancreatitis: Frequency and clinical manifestations in an Austrian patient cohort.
CFTR IVS8 Poly-T Variation Affects Severity of Acute Pancreatitis in Women.
Chronic pancreatitis associated with the p.G208A variant of PRSS1 gene in a European patient.
Chronic pancreatitis.
Clinical chronic 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.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in pancreatitis.
Cystic fibrosis transmembrane conductance regulator functional evaluations in a G542X+/- IVS8Tn:T7/9 patient with acute recurrent pancreatitis.
Cystic fibrosis transmembrane conductance regulator ion channel function testing in recurrent acute pancreatitis.
Cystic fibrosis transmembrane conductance regulator modulators reduce the risk of recurrent acute pancreatitis among adult patients with pancreas sufficient cystic fibrosis.
Early acute pancreatitis in a child with compound heterozygosis ?F508/R1438W/Y1032C cystic fibrosis: a case report.
Ethanol and its non-oxidative metabolites profoundly inhibit CFTR function in pancreatic epithelial cells which is prevented by ATP supplementation.
Genetic Evaluation of Children with Idiopathic Recurrent Acute Pancreatitis.
Genetic issues in pediatric pancreatitis.
Genetic testing in acute and chronic pancreatitis.
Genetic, epidemiological, and clinical aspects of hereditary pancreatitis: a population-based cohort study in Denmark.
Genetics and pancreatic disease.
Genetics of chronic pancreatitis.
Hereditary pancreatitis for the endoscopist.
Hereditary pancreatitis in Japan: a review of pancreatitis-associated gene mutations.
High frequency of cystic fibrosis transmembrane regulator mutation L997F in patients with recurrent idiopathic pancreatitis and in newborns with hypertrypsinemia.
Identification of CFTR, PRSS1, and SPINK1 mutations in 381 patients with pancreatitis.
Incidence, Prevalence, Etiology, and Prognosis of First-Time Chronic Pancreatitis in Young Patients: A Nationwide Cohort Study.
Is acute recurrent pancreatitis a chronic disease?
Molecular understanding of chronic pancreatitis: a perspective on the future.
Multifactorial Genesis of Pancreatitis in Primary Hyperparathyroidism: Evidence for "Protective" (PRSS2) and "Destructive" (CTRC) Genetic Factors.
Mutations in the cystic fibrosis transmembrane regulator gene in patients with tropical calcific pancreatitis.
Mutations in the SPINK1 gene in idiopathic pancreatitis Italian patients.
Mutations of the cystic fibrosis gene, but not cationic trypsinogen gene, are associated with recurrent or chronic idiopathic pancreatitis.
New advances in acute pancreatitis.
Pancreatic complications in children with cystic fibrosis.
Quantification of CFTR splice variants in adults with disseminated bronchiectasis, using the TaqMan fluorogenic detection system.
R248G cystic fibrosis transmembrane conductance regulator mutation in three siblings presenting with recurrent acute pancreatitis and reproductive issues: a case series.
Regulation of CFTR Bicarbonate Channel Activity by WNK1: Implications for Pancreatitis and CFTR-Related Disorders.
Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis.
Risk of pancreatitis with mutation of the cystic fibrosis gene.
Targeted Next-Generation Sequencing Effectively Analyzed the Cystic Fibrosis Transmembrane Conductance Regulator Gene in Pancreatitis.
The CFTR gene variants in Japanese children with idiopathic pancreatitis.
The cystic fibrosis transmembrane conductance regulator gene and ion channel function in patients with idiopathic pancreatitis.
The Importance of Aquaporin 1 in Pancreatitis and Its Relation to the CFTR Cl- Channel.
The role of pancreatic ducts in the pathogenesis of acute pancreatitis.
[Pancreatitis-associated gene mutations]
Pancreatitis, Chronic
A finger sweat chloride test for the detection of a high-risk group of chronic pancreatitis.
A novel mutation in the cystic fibrosis transmembrane conductance regulator gene in an Indian patient with idiopathic chronic pancreatitis: a case report.
Acute pancreatitis in association with Campylobacter jejuni-associated diarrhea in a 15-year-old with CFTR mutations: is there a link?
Association between F508 deletion in CFTR and chronic pancreatitis risk.
CFTR gene mutations in pancreatitis: Frequency and clinical manifestations in an Austrian patient cohort.
CFTR, PRSS1 and SPINK1 mutations in the development of pancreatitis in Brazilian patients.
CFTR, SPINK1, PRSS1, and CTRC Mutations Are Not Associated With Pancreatic Cancer in German Patients.
Chronic pancreatitis associated with the p.G208A variant of PRSS1 gene in a European patient.
Chronic pancreatitis.
Chronic pancreatitis: controversies in etiology, diagnosis and treatment.
Clinical and radiological outcome of patients suffering from chronic pancreatitis associated with gene mutations.
Clinical evidence of pathogenesis in chronic pancreatitis.
Complete cystic fibrosis transmembrane conductance regulator gene sequencing in patients with idiopathic chronic pancreatitis and controls.
Connections Between Genetics and Clinical Data: Role of MCP-1, CFTR, and SPINK-1 in the Setting of Acute, Acute Recurrent, and Chronic Pancreatitis.
Current insights into the pathogenesis of acute and chronic pancreatitis.
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-style changes in the early phase of pancreatitis.
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.
Different CFTR mutational spectrum in alcoholic and idiopathic chronic pancreatitis?
Functional characteristics of L1156F-CFTR associated with alcoholic chronic pancreatitis in Japanese.
Genetic aspects of chronic pancreatitis.
Genetic aspects of pancreatitis.
Genetics of chronic pancreatitis.
Germline mutations in CFTR and PSTI genes in chronic pancreatitis patients.
Hereditary chronic pancreatitis.
Impact of cystic fibrosis transmembrane conductance regulator gene mutation on the occurrence of chronic pancreatitis in Japanese patients.
Is acute recurrent pancreatitis a chronic disease?
Isolated idiopathic chronic pancreatitis associated with a compound heterozygosity for two mutations of the CFTR gene.
IVS8-5T Allele of CFTR is the Risk Factor in Chronic Pancreatitis, Especially in Idiopathic Chronic Pancreatitis.
Molecular understanding of chronic pancreatitis: a perspective on the future.
Motion--genetic testing is useful in the diagnosis of nonhereditary pancreatic conditions: arguments against the motion.
Motion--genetic testing is useful in the diagnosis of nonhereditary pancreatic conditions: arguments for the motion.
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.
Mutations of the cystic fibrosis gene in patients with chronic pancreatitis.
New advances in acute pancreatitis.
Pancreatic intraepithelial neoplasia is associated with chronic pancreatitis due to serine protease inhibitor kazal type 1 and cystic fibrosis transmembrane conductance regulator mutations.
Pancreatitis severity in mice with impaired CFTR function but pancreatic sufficiency is mediated via ductal and inflammatory cells-Not acinar cells.
Polymorphism of cystic fibrosis gene in Japanese patients with chronic pancreatitis.
Polyvariant mutant CFTR genes in patients with chronic pancreatitis.
Relevance of variants in serum antiproteinases for the course of chronic pancreatitis.
Spectrum of mutations and variants/haplotypes of CFTR and genotype-phenotype correlation in idiopathic chronic pancreatitis and controls in Chinese by complete analysis.
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.
[Frequency of CFTR gene mutations in idiopathic pancreatitis]
[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.
Calcium Modulated Chloride Pathways Contribute to Chloride Flux in Murine CF-Affected Macrophages.
Hypertonic saline increases lung epithelial lining fluid glutathione and thiocyanate: two protective CFTR-dependent thiols against oxidative injury.
Inflammation and CFTR: might neutrophils be the key in cystic fibrosis?
Ion channels as targets to treat cystic fibrosis lung disease.
Lack of cystic fibrosis transmembrane conductance regulator disrupts fetal airway development in pigs.
Recent advances in the understanding and treatment of cystic fibrosis.
Serum inflammatory profiles in cystic fibrosis mice with and without Bordetella pseudohinzii infection.
Sinus hypoplasia in the cystic fibrosis rat resolves in the absence of chronic infection.
Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis.
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.
Altered respiratory epithelial cell cytokine production in cystic fibrosis.
Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis.
Association of cystic fibrosis transmembrane conductance regulator gene variants with acute lung injury in African American children with 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.
Cystic fibrosis transmembrane conductance regulator regulates epithelial cell response to Aspergillus and resultant pulmonary inflammation.
Markers of early disease and prognosis in COPD.
Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth.
Reduced GM1 Ganglioside in CFTR-Deficient Human Airway Cells Results in Decreased ss1-integrin Signaling and Delayed Wound Repair.
Role of CD95 in pulmonary inflammation and infection in cystic fibrosis.
Role of IL-10 deficiency in excessive nuclear factor-kappaB activation and lung inflammation in cystic fibrosis transmembrane conductance regulator knockout mice.
Systemic inflammatory mediators and cystic fibrosis genotype.
Polycystic Kidney Diseases
ABSOLUTE CONFIGURATION AND BIOLOGICAL PROPERTIES OF ENANTIOMERS OF CFTR INHIBITOR BPO-27.
Activation of AMP-activated kinase as a strategy for managing autosomal dominant polycystic kidney disease.
Alpha-aminoazaheterocyclic-methylglyoxal adducts do not inhibit cystic fibrosis transmembrane conductance regulator chloride channel activity.
Basolateral chloride transporters in autosomal dominant polycystic kidney disease.
Benzopyrimido-pyrrolo-oxazine-dione (R)-BPO-27 Inhibits CFTR Chloride Channel Gating by Competition with ATP.
CF gene and cystic fibrosis transmembrane conductance regulator expression in autosomal dominant polycystic kidney disease.
CFTR Inhibitors.
Cystic fibrosis transmembrane conductance regulator chloride channel blockers: Pharmacological, biophysical and physiological relevance.
Exome sequencing of Saudi Arabian patients with ADPKD.
Identification of resveratrol oligomers as inhibitors of cystic fibrosis transmembrane conductance regulator by high-throughput screening of natural products from chinese medicinal plants.
Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth.
Inhibition of histone deacetylase 6 activity reduces cyst growth in polycystic kidney disease.
Lipid Peroxidation Drives Renal Cyst Growth In Vitro through Activation of TMEM16A.
Mechanism and application of metformin in kidney diseases: An update.
Nanomolar Potency Pyrimido-pyrrolo-quinoxalinedione CFTR Inhibitor Reduces Cyst Size in a Polycystic Kidney Disease Model.
Novel action of the chalcone isoliquiritigenin as a cystic fibrosis transmembrane conductance regulator (CFTR) inhibitor: potential therapy for cholera and polycystic kidney disease.
Novel Action of the Chalcone Isoliquiritigenin as a Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Inhibitor: Potential Therapy for Cholera and Polycystic Kidney Disease.
Potent, Metabolically Stable Benzopyrimido-pyrrolo-oxazine-dione (BPO) CFTR Inhibitors for Polycystic Kidney Disease.
Role of CFTR in autosomal recessive polycystic kidney disease.
Small-molecule CFTR inhibitors slow cyst growth in polycystic kidney disease.
Steviol Reduces MDCK Cyst Formation and Growth by Inhibiting CFTR Channel Activity and Promoting Proteasome-Mediated CFTR Degradation.
Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease.
The cystic fibrosis transmembrane conductance regulator mediates transepithelial fluid secretion by human autosomal dominant polycystic kidney disease epithelium in vitro.
The relationship between cell proliferation, Cl- secretion, and renal cyst growth: a study using CFTR inhibitors.
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.
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.
A role for CFTR in human autosomal dominant polycystic kidney disease.
Activation of AMP-activated kinase as a strategy for managing autosomal dominant polycystic kidney disease.
Autosomal dominant polycystic kidney disease coexisting with cystic fibrosis.
Basolateral chloride transporters in autosomal dominant polycystic kidney disease.
cAMP regulates cell proliferation and cyst formation in autosomal polycystic kidney disease cells.
CF gene and cystic fibrosis transmembrane conductance regulator expression in autosomal dominant polycystic kidney disease.
CFTR Inhibitors.
Exome sequencing of Saudi Arabian patients with ADPKD.
Fluid transport and cystogenesis in autosomal dominant polycystic kidney disease.
Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth.
Inhibition of histone deacetylase 6 activity reduces cyst growth in polycystic kidney disease.
Mechanism and application of metformin in kidney diseases: An update.
Role of CFTR in autosomal recessive polycystic kidney disease.
Steviol retards renal cyst growth through reduction of CFTR expression and inhibition of epithelial cell proliferation in a mouse model of polycystic kidney disease.
Targeting chloride transport in autosomal dominant polycystic kidney disease.
The cystic fibrosis transmembrane conductance regulator mediates transepithelial fluid secretion by human autosomal dominant polycystic kidney disease epithelium in vitro.
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.
Therapeutic potential of cystic fibrosis transmembrane conductance regulator (CFTR) inhibitors in polycystic kidney disease.
[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.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene 5T allele may protect against prostate cancer: a case-control study in Chinese Han population.
Knockdown of CFTR enhances sensitivity of prostate cancer cells to cisplatin via inhibition of autophagy.
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.
Airway surface liquid composition in mice.
Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator.
CFTR mutations and host susceptibility to Pseudomonas aeruginosa lung infection.
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.
Influence of cystic fibrosis transmembrane conductance regulator on gene expression in response to Pseudomonas aeruginosa infection of human bronchial epithelial cells.
Mucociliary clearance in cystic fibrosis knockout mice infected with Pseudomonas aeruginosa.
Pathophysiology and management of pulmonary infections in cystic fibrosis.
Role for cystic fibrosis transmembrane conductance regulator protein in a glutathione response to bronchopulmonary pseudomonas infection.
Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections.
Specific resistance to Pseudomonas aeruginosa infection in zebrafish is mediated by the cystic fibrosis transmembrane conductance regulator.
Variants in the glutamate-cysteine-ligase gene are associated with cystic fibrosis lung disease.
Pulmonary Alveolar Proteinosis
Aerosolized Medications for Gene and Peptide Therapy.
Pulmonary Arterial Hypertension
Autophagy and inflammation in chronic respiratory disease.
Pulmonary Disease, Chronic Obstructive
Acquired defects in CFTR-dependent ?-adrenergic sweat secretion in chronic obstructive pulmonary disease.
Assessing effects of inhaled antibiotics in adults with non-cystic fibrosis bronchiectasis--experiences from recent clinical trials.
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.
Autophagy and inflammation in chronic respiratory disease.
CFTR dysfunction in cystic fibrosis and chronic obstructive pulmonary disease.
CFTR gene mutations--including three novel nucleotide substitutions--and haplotype background in patients with asthma, disseminated bronchiectasis and chronic obstructive pulmonary disease.
Cigarette Smoke-Induced Acquired Dysfunction of Cystic Fibrosis Transmembrane Conductance Regulator in the Pathogenesis of Chronic Obstructive Pulmonary Disease.
Correlation of apical fluid-regulating channel proteins with lung function in human COPD lungs.
Cystic Fibrosis Transmembrane Conductance Regulator. Implications in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Discovery of Icenticaftor (QBW251), a Cystic Fibrosis Transmembrane Conductance Regulator Potentiator with Clinical Efficacy in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Effect of lentivirus-mediated CFTR overexpression on oxidative stress injury and inflammatory response in the lung tissue of COPD mouse model.
Genetic and biochemical markers of obstructive lung disease in the general population.
Markers of early disease and prognosis in COPD.
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.
TGF-beta 1 and Cigarette Smoke Inhibit the Ability of ?2-agonists to Enhance Epithelial Permeability.
The mucus bundles responsible for airway cleaning are retained in cystic fibrosis and by cholinergic stimulation.
The search for susceptibility genes of COPD.
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.
Cytokine-Regulation of Na(+)-K(+)-Cl(-) Cotransporter 1 and Cystic Fibrosis Transmembrane Conductance Regulator-Potential Role in Pulmonary Inflammation and Edema Formation.
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.
Regulation of the Coxsackie and adenovirus receptor expression is dependent on cystic fibrosis transmembrane regulator in airway epithelial cells.
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
Autophagy and inflammation in chronic respiratory disease.
Effects of antiallergic herbal agents on cystic fibrosis transmembrane conductance regulator in nasal mucosal epithelia of allergic rhinitis rabbits.
Sarcoidosis
Aerosolized Medications for Gene and Peptide Therapy.
CFTR gene mutations in sarcoidosis.
Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease.
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.
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.
Cystic fibrosis gene mutations deltaF508 and 394delTT in patients with chronic sinusitis in Finland.
Effect of L-ascorbate on chloride transport in freshly excised sinonasal epithelia.
Maxillary sinusitis as a surrogate model for CF gene therapy clinical trials in patients with antrostomies.
Multi-dimensional clinical phenotyping of a national cohort of adult cystic fibrosis patients.
Sleep Apnea, Obstructive
Autophagy and inflammation in chronic respiratory disease.
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.
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
ATP-binding cassette (ABC) transporters in normal and pathological lung.
Inhibitors of ATP-binding cassette transporters suppress interleukin-12 p40 production and major histocompatibility complex II up-regulation in macrophages.
Tuberculosis
Aerosolized Medications for Gene and Peptide Therapy.
Autophagy and inflammation in chronic respiratory disease.
Tuberous Sclerosis
Exome sequencing of Saudi Arabian patients with ADPKD.
Typhoid Fever
Structural basis of typhoid: Salmonella typhi type IVb pilin (PilS) and cystic fibrosis transmembrane conductance regulator interaction.
Susceptibility to typhoid fever is associated with a polymorphism in the cystic fibrosis transmembrane conductance regulator (CFTR).
Urogenital Abnormalities
[Congenital absence of the vas deferens]
Uterine Cervical Neoplasms
CFTR Regulates the Proliferation, Migration and Invasion of Cervical Cancer Cells by Inhibiting the NF-?B Signalling Pathway.
Constitutive activation of nuclear factor ?B contributes to cystic fibrosis transmembrane conductance regulator expression and promotes human cervical cancer progression and poor prognosis.
Overexpression of cystic fibrosis transmembrane conductance regulator (CFTR) is associated with human cervical cancer malignancy, progression and prognosis.
Varicocele
[AFU and SALF recommendations for the evaluation of male infertility].
Virus Diseases
Influenza virus M2 targets cystic fibrosis transmembrane conductance regulator for lysosomal degradation during viral infection.
The new frontier: gene and oligonucleotide therapy.
Whooping Cough
G-protein regulation of outwardly rectified epithelial chloride channels incorporated into planar bilayer membranes.
Mobilization of intracellular Ca2+ and stimulation of cyclic AMP production by kappa opioid receptors expressed in Xenopus oocytes.
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.