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Disease on EC 3.6.4.7 - peroxisome-assembly ATPase

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Acquired Immunodeficiency Syndrome
Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation.
Adenocarcinoma
Increased expression of valosin-containing protein (p97) is associated with lymph node metastasis and prognosis of pancreatic ductal adenocarcinoma.
Adrenoleukodystrophy
Organelle disease: peroxisomal disorders.
Alzheimer Disease
Identification of a novel valosin-containing protein polymorphism in late-onset Alzheimer's disease.
Amyotrophic Lateral Sclerosis
A patient with familial amyotrophic lateral sclerosis associated with a new valosin-containing protein (VCP) gene mutation.
Altered intracellular localization and valosin-containing protein (p97 VCP) interaction underlie ATP7A-related distal motor neuropathy.
An autopsy report of a familial amyotrophic lateral sclerosis case carrying VCP Arg487His mutation with a unique TDP43 proteinopathy.
Ceramide contributes to pathogenesis and may be targeted for therapy in VCP inclusion body myopathy.
Characterization of the Asian myopathy patients with VCP mutations.
Erratum: A patient with familial amyotrophic lateral sclerosis associated with a new valosin-containing protein (VCP) gene mutation.
Exome sequencing reveals VCP mutations as a cause of familial ALS.
Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant.
Increased expression of valosin-containing protein in the skin of patients with amyotrophic lateral sclerosis.
Lipid-enriched diet rescues lethality and slows down progression in a murine model of VCP-associated disease.
mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy.
Mutational analysis of VCP gene in familial amyotrophic lateral sclerosis.
Novel valosin-containing protein mutations associated with multisystem proteinopathy.
Novel VCP mutations expand the mutational spectrum of frontotemporal dementia.
Screening of VCP mutations in Chinese amyotrophic lateral sclerosis patients.
SVIP is a molecular determinant of lysosomal dynamic stability, neurodegeneration and lifespan.
Valosin-containing protein (VCP) mutations in sporadic amyotrophic lateral sclerosis.
VCP mutations in familial and sporadic amyotrophic lateral sclerosis.
[The molecular pathology of frontotemporal lobar degeneration]
Astrocytoma
Enterovirus 71 induces neural cell apoptosis and autophagy through promoting ACOX1 downregulation and ROS generation.
Blindness
PEX1 deficiency presenting as Leber congenital amaurosis.
Breast Neoplasms
High expression of valosin-containing protein predicts poor prognosis in patients with breast carcinoma.
p97/VCP is highly expressed in the stem-like cells of breast cancer and controls cancer stemness partly through the unfolded protein response.
Carcinogenesis
Epigenetic loss of the endoplasmic reticulum-associated degradation inhibitor SVIP induces cancer cell metabolic reprogramming.
Lon Peptidase 2, Peroxisomal (LONP2) Contributes to Cervical Carcinogenesis via Oxidative Stress.
Neurotrophin 3/TrkC-regulated proteins in the human medulloblastoma cell line DAOY.
Subcellular and functional proteomic analysis of the cellular responses induced by Helicobacter pylori.
VCP phosphorylation-dependent interaction partners prevent apoptosis in Helicobacter pylori-infected gastric epithelial cells.
Carcinoma
Expression level of valosin-containing protein (p97) is associated with prognosis of esophageal carcinoma.
Expression level of valosin-containing protein (p97) is correlated with progression and prognosis of non-small-cell lung carcinoma.
Expression level of valosin-containing protein (VCP) as a prognostic marker for gingival squamous cell carcinoma.
Expression level of valosin-containing protein is strongly associated with progression and prognosis of gastric carcinoma.
Prognostic significance of pre B cell leukemia transcription factor 2 (PBX2) expression in non-small cell lung carcinoma.
Targeting valosin-containing protein enhances the efficacy of radiation therapy in esophageal squamous cell carcinoma.
Valosin-containing protein (VCP/p97)-expression correlates with prognosis of HPV- negative oropharyngeal squamous cell carcinoma (OSCC).
Carcinoma, Hepatocellular
Elevated expression of valosin-containing protein (p97) in hepatocellular carcinoma is correlated with increased incidence of tumor recurrence.
Carcinoma, Non-Small-Cell Lung
Expression level of valosin-containing protein (p97) is correlated with progression and prognosis of non-small-cell lung carcinoma.
Carcinoma, Squamous Cell
Expression level of valosin-containing protein (VCP) as a prognostic marker for gingival squamous cell carcinoma.
Cardiomegaly
Novel genomic targets of valosin-containing protein in protecting pathological cardiac hypertrophy.
Cardiomyopathies
Disruption of valosin-containing protein activity causes cardiomyopathy and reveals pleiotropic functions in cardiac homeostasis.
Cataract
Variant analysis of PEX11B gene from a family with peroxisome biogenesis disorder 14B by whole exome sequencing.
Choriocarcinoma
Disruption of p97/VCP induces autophagosome accumulation, cell cycle arrest and apoptosis in human choriocarcinoma cells.
Cockayne Syndrome
Valosin-containing Protein (VCP)/p97 Segregase Mediates Proteolytic Processing of Cockayne Syndrome Group B (CSB) in Damaged Chromatin.
Colorectal Neoplasms
A covalent p97/VCP ATPase inhibitor can overcome resistance to CB-5083 and NMS-873 in colorectal cancer cells.
Expression of valosin-containing protein in colorectal carcinomas as a predictor for disease recurrence and prognosis.
Valosin-containing protein (VCP) promotes the growth, invasion, and metastasis of colorectal cancer through activation of STAT3 signaling.
Communicable Diseases
Infectious disease: Peroxin inhibitor treats Trypanosoma infection.
Congenital Abnormalities
Peroxin 5: a cycling receptor for protein translocation into peroxisomes.
Dementia
Altered intracellular localization and valosin-containing protein (p97 VCP) interaction underlie ATP7A-related distal motor neuropathy.
Amyotrophic lateral sclerosis, frontotemporal lobar dementia, and p62: A functional convergence?
Brain imaging and neuropsychology in late-onset dementia due to a novel mutation (R93C) of valosin-containing protein.
Distal myopathy and rapidly progressive dementia associated with a novel mutation in the VCP gene: Expanding inclusion body myopathy with early-onset Paget disease and frontotemporal dementia spectrum.
Identification of a novel valosin-containing protein polymorphism in late-onset Alzheimer's disease.
Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy.
Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation.
Late-onset autosomal dominant limb girdle muscular dystrophy and Paget's disease of bone unlinked to the VCP gene locus.
Mechanisms of disease: genetics of Paget's disease of bone and related disorders.
mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy.
Mutational analysis of the VCP gene in Parkinson's disease.
Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia.
[An advanced case of myopathy and dementia with a new mutation in the valosin-containing protein gene].
Distal Myopathies
Distal myopathy and rapidly progressive dementia associated with a novel mutation in the VCP gene: Expanding inclusion body myopathy with early-onset Paget disease and frontotemporal dementia spectrum.
Encephalitis, Japanese
Valosin-containing protein/p97 plays critical roles in the Japanese encephalitis virus life cycle.
Esophageal Squamous Cell Carcinoma
Targeting valosin-containing protein enhances the efficacy of radiation therapy in esophageal squamous cell carcinoma.
Fetal Growth Retardation
Peroxisome Deficiency Dysregulates Fatty Acid Oxidization and Exacerbates Lipotoxicity in ? Cells.
Frontotemporal Dementia
A cross-sectional analysis of clinical evaluation in 35 individuals with mutations of the valosin-containing protein gene.
A novel exon 2 I27V VCP variant is associated with dissimilar clinical syndromes.
A novel mutation in the VCP gene (G157R) in a German family with inclusion-body myopathy with Paget disease of bone and frontotemporal dementia.
A progressive translational mouse model of human valosin-containing protein disease: the VCP(R155H/+) mouse.
A unique IBMPFD-related P97/VCP mutation with differential binding pattern and subcellular localization.
Altered cofactor regulation with disease-associated p97/VCP mutations.
An Italian family with inclusion-body myopathy and frontotemporal dementia due to mutation in the VCP gene.
Another VCP interactor: NF is enough.
APOE is a potential modifier gene in an autosomal dominant form of frontotemporal dementia (IBMPFD).
ATP-bound form of the D1 AAA domain inhibits an essential function of Cdc48p/p97.
Axonal hyperpolarization in inclusion-body myopathy, Paget disease of the bone, and frontotemporal dementia (IBMPFD).
Brain imaging and neuropsychology in late-onset dementia due to a novel mutation (R93C) of valosin-containing protein.
Ceramide contributes to pathogenesis and may be targeted for therapy in VCP inclusion body myopathy.
Characterization of the Asian myopathy patients with VCP mutations.
Clinical outcome in 19 French and Spanish patients with valosin-containing protein myopathy associated with Paget's disease of bone and frontotemporal dementia.
Distinct distal myopathy phenotype caused by VCP gene mutation in a Finnish family.
Early-onset Alzheimers and Cortical Vision Impairment in a Woman With Valosin-containing Protein Disease Associated With 2 APOE [Latin Small Letter Open E]4/APOE [Latin Small Letter Open E]4 Genotype.
Etiologic factors in Paget's disease of bone.
Global Gene Expression Profiling in R155H Knock-In Murine Model of VCP Disease.
Global gene profiling of VCP-associated inclusion body myopathy.
Hereditary inclusion body myopathy-linked p97/VCP mutations in the NH2 domain and the D1 ring modulate p97/VCP ATPase activity and D2 ring conformation.
Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant.
Impaired protein aggregate handling and clearance underlie the pathogenesis of p97/VCP-associated disease.
Inclusion body myopathy and frontotemporal dementia caused by a novel VCP mutation.
Inclusion body myopathy and Paget disease is linked to a novel mutation in the VCP gene.
Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein.
Inclusion Body Myopathy With Paget Disease of Bone and Frontotemporal Dementia Linked to VCP p.Arg155Cys in a Korean Family.
Involvement of peripheral and central nervous systems in a valosin-containing protein mutation.
Juvenile Paget's disease, familial expansile osteolysis and other genetic osteolytic disorders.
Lipid-enriched diet rescues lethality and slows down progression in a murine model of VCP-associated disease.
Multisystem proteinopathy: Where myopathy and motor neuron disease converge.
Mutant valosin-containing protein causes a novel type of frontotemporal dementia.
Mutational analysis of VCP gene in familial amyotrophic lateral sclerosis.
Neuronal-specific overexpression of a mutant valosin-containing protein associated with IBMPFD promotes aberrant ubiquitin and TDP-43 accumulation and cognitive dysfunction in transgenic mice.
Novel ubiquitin neuropathology in frontotemporal dementia with valosin-containing protein gene mutations.
Novel valosin-containing protein mutations associated with multisystem proteinopathy.
Novel VCP mutations expand the mutational spectrum of frontotemporal dementia.
Novel VCP mutations in inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia.
Nuclear inclusions mimicking poly(A)-binding protein nuclear 1 inclusions in a case of inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia with a novel mutation in the valosin-containing protein gene.
Nuclear localization of valosin-containing protein in normal muscle and muscle affected by inclusion-body myositis.
Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model.
Pathological consequences of VCP mutations on human striated muscle.
Phenotypic diversity in an international Cure VCP Disease registry.
Phenotypic variability in three families with valosin-containing protein mutation.
Psychiatric Presentation of Frontotemporal Dementia Associated with Inclusion Body Myopathy due to the VCP Mutation (R155H) in a French Family.
Radiological features of Paget disease of bone associated with VCP myopathy.
Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice.
Structural and functional deviations in disease-associated p97 mutants.
Strumpellin is a novel valosin-containing protein binding partner linking hereditary spastic paraplegia to protein aggregation diseases.
Targeted Excision of VCP R155H Mutation by Cre-LoxP Technology as a Promising Therapeutic Strategy for Valosin-Containing Protein Disease.
TDP-43 in Familial and Sporadic Frontotemporal Lobar Degeneration with Ubiquitin Inclusions.
TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions.
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations.
TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.
The Myoblast C2C12 Transfected with Mutant Valosin-Containing Protein Exhibits Delayed Stress Granule Resolution on Oxidative Stress.
Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice.
Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.
Valosin-containing protein (VCP) is a novel IQ motif-containing GTPase activating protein 1 (IQGAP1)-interacting protein.
Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease.
Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy.
VCP gene analyses in Japanese patients with sporadic amyotrophic lateral sclerosis identify a new mutation.
[A case of inclusion body myopathy with Paget's disease of bone and frontotemporal dementia (IBMPFD) showing clinical features of motor neuron disease].
[Inclusion body myositis, Paget's disease of the bone and frontotemporal dementia: early involvement of the heart and respiratory muscles].
[Multilocular Paget's disease in IBMPFD syndrome. A case report with 14-year follow-up].
[The molecular pathology of frontotemporal lobar degeneration]
Frontotemporal Lobar Degeneration
ALS and FTLD: two faces of TDP-43 proteinopathy.
Amyotrophic lateral sclerosis, frontotemporal lobar dementia, and p62: A functional convergence?
Clinic, neuropathology and molecular genetics of frontotemporal dementia: a mini-review.
Clinical heterogeneity in 3 unrelated families linked to VCP p.Arg159His.
Frontotemporal dementias: update on recent developments in molecular genetics and neuropathology.
Recent advances in the genetics of the ALS-FTLD complex.
The molecular genetics and neuropathology of frontotemporal lobar degeneration: recent developments.
[Frontotemporal dementia (FTD) and genetic mutations including progranulin gene]
[The molecular pathology of frontotemporal lobar degeneration]
Genetic Diseases, Inborn
Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice.
Glaucoma
Effect of VCP modulators on gene expression profiles of retinal ganglion cells in an acute injury mouse model.
Valosin-Containing Protein is a Novel Autoantigen in Patients with Glaucoma.
Glioblastoma
Retinoid receptor turnover mediated by sumoylation, ubiquitination and the valosin-containing protein is disrupted in glioblastoma.
Glioma
Valosin-containing protein regulates the proteasome-mediated degradation of DNA-PKcs in glioma cells.
Hearing Loss
Variant analysis of PEX11B gene from a family with peroxisome biogenesis disorder 14B by whole exome sequencing.
Hepatitis B
Hepatitis B virus X protein enhances activation of nuclear factor ?B through interaction with valosin-containing protein.
Hepatitis C
Affinity purification of the Hepatitis C virus replicase identifies Valosin-containing protein (VCP) AAA+ATPase as an active viral replication modulator.
Aggregation of a hepatitis C virus replicase module induced by ablation of p97/VCP.
Huntington Disease
Enhanced ATPase activities as a primary defect of mutant valosin-containing proteins that cause inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia.
Infections
Colletotrichum orbiculare FAM1 Encodes a Novel Woronin Body-Associated Pex22 Peroxin Required for Appressorium-Mediated Plant Infection.
Enterovirus 71 induces neural cell apoptosis and autophagy through promoting ACOX1 downregulation and ROS generation.
Genome-Wide Screen Reveals Valosin-Containing Protein Requirement for Coronavirus Exit from Endosomes.
Infectious disease: Peroxin inhibitor treats Trypanosoma infection.
MrPEX33 is involved in infection-related morphogenesis and pathogenicity of Metarhizium robertsii.
Intellectual Disability
Variant analysis of PEX11B gene from a family with peroxisome biogenesis disorder 14B by whole exome sequencing.
Ischemic Stroke
KUS121, a valosin-containing protein modulator, attenuates ischemic stroke via preventing ATP depletion.
Leber Congenital Amaurosis
PEX1 deficiency presenting as Leber congenital amaurosis.
Leishmaniasis
Down-regulation of peroxin synthesis by silencing RNA (siRNA): A novel hypothesis for treatment of leishmaniasis.
Leukemia
Prognostic significance of pre B cell leukemia transcription factor 2 (PBX2) expression in non-small cell lung carcinoma.
Leukemia, Myeloid, Acute
Targeting acute myeloid leukemia dependency on VCP-mediated DNA repair through a selective second-generation small-molecule inhibitor.
Leukemia, T-Cell
What's in the Literature?
Liposarcoma
Monocytes of patients with amyotrophic lateral sclerosis linked to gene mutations display altered TDP-43 subcellular distribution.
Liver Cirrhosis, Biliary
Autoantibodies from primary biliary cirrhosis patients with anti-p95c antibodies bind to recombinant p97/VCP and inhibit in vitro nuclear envelope assembly.
Liver Neoplasms
Sorafenib-mediated targeting of the AAA? ATPase p97/VCP leads to disruption of the secretory pathway, endoplasmic reticulum stress, and hepatocellular cancer cell death.
Lymphatic Metastasis
Increased expression of valosin-containing protein (p97) is associated with lymph node metastasis and prognosis of pancreatic ductal adenocarcinoma.
Lon Peptidase 2, Peroxisomal (LONP2) Contributes to Cervical Carcinogenesis via Oxidative Stress.
Lymphoma
Expression level of valosin containing protein is associated with prognosis of primary orbital MALT lymphoma.
In vitro efficacy of a first-generation valosin-containing protein inhibitor (CB-5083) against canine lymphoma.
Lymphoma, B-Cell
Upregulation of valosin-containing protein (VCP) is associated with poor prognosis and promotes tumor progression of orbital B-cell lymphoma.
Machado-Joseph Disease
Enhanced ATPase activities as a primary defect of mutant valosin-containing proteins that cause inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia.
Melanoma
Exposure to Melan-A/MART-126-35 tumor epitope specific CD8(+)T cells reveals immune escape by affecting the ubiquitin-proteasome system (UPS).
Motor Neuron Disease
Altered intracellular localization and valosin-containing protein (p97 VCP) interaction underlie ATP7A-related distal motor neuropathy.
ATP7A trafficking and mechanisms underlying the distal motor neuropathy induced by mutations in ATP7A.
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies.
Multiple Myeloma
Novel p97/VCP inhibitor induces endoplasmic reticulum stress and apoptosis in both bortezomib-sensitive and -resistant multiple myeloma cells.
Muscle Weakness
Global Gene Expression Profiling in R155H Knock-In Murine Model of VCP Disease.
Muscular Atrophy
The p97/VCP ATPase is critical in muscle atrophy and the accelerated degradation of muscle proteins.
Muscular Diseases
A cross-sectional analysis of clinical evaluation in 35 individuals with mutations of the valosin-containing protein gene.
A novel mutation in the VCP gene (G157R) in a German family with inclusion-body myopathy with Paget disease of bone and frontotemporal dementia.
A progressive translational mouse model of human valosin-containing protein disease: the VCP(R155H/+) mouse.
A unique IBMPFD-related P97/VCP mutation with differential binding pattern and subcellular localization.
Activation of the NLRP3 Inflammasome Is Associated with Valosin-Containing Protein Myopathy.
Altered cofactor regulation with disease-associated p97/VCP mutations.
Altered intracellular localization and valosin-containing protein (p97 VCP) interaction underlie ATP7A-related distal motor neuropathy.
An Italian family with inclusion-body myopathy and frontotemporal dementia due to mutation in the VCP gene.
Another VCP interactor: NF is enough.
APOE is a potential modifier gene in an autosomal dominant form of frontotemporal dementia (IBMPFD).
ATP-bound form of the D1 AAA domain inhibits an essential function of Cdc48p/p97.
Axonal hyperpolarization in inclusion-body myopathy, Paget disease of the bone, and frontotemporal dementia (IBMPFD).
Brain imaging and neuropsychology in late-onset dementia due to a novel mutation (R93C) of valosin-containing protein.
Ceramide contributes to pathogenesis and may be targeted for therapy in VCP inclusion body myopathy.
Characterization of the Asian myopathy patients with VCP mutations.
Clinical heterogeneity in 3 unrelated families linked to VCP p.Arg159His.
Clinical outcome in 19 French and Spanish patients with valosin-containing protein myopathy associated with Paget's disease of bone and frontotemporal dementia.
Crucial mitochondrial impairment upon CDC48 mutation in apoptotic yeast.
Distal myopathy and rapidly progressive dementia associated with a novel mutation in the VCP gene: Expanding inclusion body myopathy with early-onset Paget disease and frontotemporal dementia spectrum.
Distinct distal myopathy phenotype caused by VCP gene mutation in a Finnish family.
Early-onset Alzheimers and Cortical Vision Impairment in a Woman With Valosin-containing Protein Disease Associated With 2 APOE [Latin Small Letter Open E]4/APOE [Latin Small Letter Open E]4 Genotype.
Etiologic factors in Paget's disease of bone.
Global Gene Expression Profiling in R155H Knock-In Murine Model of VCP Disease.
Global gene profiling of VCP-associated inclusion body myopathy.
Hereditary inclusion body myopathy-linked p97/VCP mutations in the NH2 domain and the D1 ring modulate p97/VCP ATPase activity and D2 ring conformation.
Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant.
Impaired protein aggregate handling and clearance underlie the pathogenesis of p97/VCP-associated disease.
Inclusion body myopathy and frontotemporal dementia caused by a novel VCP mutation.
Inclusion body myopathy and Paget disease is linked to a novel mutation in the VCP gene.
Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein.
Inclusion Body Myopathy With Paget Disease of Bone and Frontotemporal Dementia Linked to VCP p.Arg155Cys in a Korean Family.
Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy.
Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation.
Involvement of peripheral and central nervous systems in a valosin-containing protein mutation.
Juvenile Paget's disease, familial expansile osteolysis and other genetic osteolytic disorders.
Lipid-enriched diet rescues lethality and slows down progression in a murine model of VCP-associated disease.
Mechanisms of disease: genetics of Paget's disease of bone and related disorders.
Mitochondrial Respiratory Measurements in Patient-derived Fibroblasts.
mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy.
Multisystem proteinopathy: Where myopathy and motor neuron disease converge.
Mutant valosin-containing protein causes a novel type of frontotemporal dementia.
Mutational analysis of VCP gene in familial amyotrophic lateral sclerosis.
Neuronal-specific overexpression of a mutant valosin-containing protein associated with IBMPFD promotes aberrant ubiquitin and TDP-43 accumulation and cognitive dysfunction in transgenic mice.
Novel ubiquitin neuropathology in frontotemporal dementia with valosin-containing protein gene mutations.
Novel valosin-containing protein mutations associated with multisystem proteinopathy.
Novel VCP mutations expand the mutational spectrum of frontotemporal dementia.
Novel VCP mutations in inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia.
Nuclear inclusions mimicking poly(A)-binding protein nuclear 1 inclusions in a case of inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia with a novel mutation in the valosin-containing protein gene.
Nuclear localization of valosin-containing protein in normal muscle and muscle affected by inclusion-body myositis.
Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model.
Pathological consequences of VCP mutations on human striated muscle.
Phenotypic diversity in an international Cure VCP Disease registry.
Phenotypic variability in three families with valosin-containing protein mutation.
Psychiatric Presentation of Frontotemporal Dementia Associated with Inclusion Body Myopathy due to the VCP Mutation (R155H) in a French Family.
Radiological features of Paget disease of bone associated with VCP myopathy.
Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice.
Structural and functional deviations in disease-associated p97 mutants.
Strumpellin is a novel valosin-containing protein binding partner linking hereditary spastic paraplegia to protein aggregation diseases.
Targeted Excision of VCP R155H Mutation by Cre-LoxP Technology as a Promising Therapeutic Strategy for Valosin-Containing Protein Disease.
Targeting protein homeostasis in sporadic inclusion body myositis.
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations.
TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.
The Myoblast C2C12 Transfected with Mutant Valosin-Containing Protein Exhibits Delayed Stress Granule Resolution on Oxidative Stress.
Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice.
Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.
Translocation of molecular chaperones to the titin springs is common in skeletal myopathy patients and affects sarcomere function.
Valosin-containing protein (VCP) is a novel IQ motif-containing GTPase activating protein 1 (IQGAP1)-interacting protein.
Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease.
Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy.
Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia.
VCP gene analyses in Japanese patients with sporadic amyotrophic lateral sclerosis identify a new mutation.
[A case of inclusion body myopathy with Paget's disease of bone and frontotemporal dementia (IBMPFD) showing clinical features of motor neuron disease].
[An advanced case of myopathy and dementia with a new mutation in the valosin-containing protein gene].
[Multilocular Paget's disease in IBMPFD syndrome. A case report with 14-year follow-up].
Muscular Dystrophies
Late-onset autosomal dominant limb girdle muscular dystrophy and Paget's disease of bone unlinked to the VCP gene locus.
SVIP is a molecular determinant of lysosomal dynamic stability, neurodegeneration and lifespan.
Muscular Dystrophy, Duchenne
Translocation of molecular chaperones to the titin springs is common in skeletal myopathy patients and affects sarcomere function.
Myositis
A novel exon 2 I27V VCP variant is associated with dissimilar clinical syndromes.
Nuclear localization of valosin-containing protein in normal muscle and muscle affected by inclusion-body myositis.
Translocation of molecular chaperones to the titin springs is common in skeletal myopathy patients and affects sarcomere function.
What's in the Literature?
[Inclusion body myositis, Paget's disease of the bone and frontotemporal dementia: early involvement of the heart and respiratory muscles].
Myositis, Inclusion Body
A novel exon 2 I27V VCP variant is associated with dissimilar clinical syndromes.
Translocation of molecular chaperones to the titin springs is common in skeletal myopathy patients and affects sarcomere function.
What's in the Literature?
[Inclusion body myositis, Paget's disease of the bone and frontotemporal dementia: early involvement of the heart and respiratory muscles].
Neoplasm Metastasis
Demethylation-mediated miR-129-5p up-regulation inhibits malignant phenotype of osteogenic osteosarcoma by targeting Homo sapiens valosin-containing protein (VCP).
E74-like factor 2 regulates valosin-containing protein expression.
Elevated expression of valosin-containing protein (p97) in hepatocellular carcinoma is correlated with increased incidence of tumor recurrence.
Elevated expression of valosin-containing protein (p97) is associated with poor prognosis of prostate cancer.
Expression level of valosin-containing protein (p97) is associated with prognosis of esophageal carcinoma.
Expression level of valosin-containing protein (p97) is correlated with progression and prognosis of non-small-cell lung carcinoma.
Expression level of valosin-containing protein (VCP) as a prognostic marker for gingival squamous cell carcinoma.
Expression level of valosin-containing protein is strongly associated with progression and prognosis of gastric carcinoma.
Expression of valosin-containing protein in colorectal carcinomas as a predictor for disease recurrence and prognosis.
Identification and characterization of valosin-containing protein (VCP/p97) in untransformed osteoblast-like cells.
Increased expression of valosin-containing protein (p97) is associated with lymph node metastasis and prognosis of pancreatic ductal adenocarcinoma.
Inhibiting valosin-containing protein suppresses osteosarcoma cell metastasis via AKT/nuclear factor of kappa B signaling pathway in vitro.
Lon Peptidase 2, Peroxisomal (LONP2) Contributes to Cervical Carcinogenesis via Oxidative Stress.
Prognostic significance of pre B cell leukemia transcription factor 2 (PBX2) expression in non-small cell lung carcinoma.
Valosin-containing protein (VCP) promotes the growth, invasion, and metastasis of colorectal cancer through activation of STAT3 signaling.
Valosin-containing protein promotes metastasis of osteosarcoma through autophagy induction and anoikis inhibition via the ERK/NF-??/beclin-1 signaling pathway.
Neoplasms
A Dynamic molecular basis for malfunction in disease mutants of p97/VCP.
A novel function of AAA-ATPase p97/VCP in the regulation of cell motility.
A p97/Valosin-Containing Protein Inhibitor Drug CB-5083 Has a Potent but Reversible Off-Target Effect on Phosphodiesterase-6.
Allosteric p97 inhibitors can overcome resistance to ATP-competitive p97 inhibitors for potential anti-cancer therapy.
ATPase activity of p97/valosin-containing protein is regulated by oxidative modification of the evolutionally conserved cysteine 522 residue in Walker A motif.
Crystal structure of human FAF1 UBX domain reveals a novel FcisP touch-turn motif in p97/VCP-binding region.
Disruption of p97/VCP induces autophagosome accumulation, cell cycle arrest and apoptosis in human choriocarcinoma cells.
E74-like factor 2 regulates valosin-containing protein expression.
E74-like factor 2 transactivates valosin-containing protein gene, a gene involved in cancer growth.
Eeyarestatin I derivatives with improved aqueous solubility.
Elevated expression of valosin-containing protein (p97) in hepatocellular carcinoma is correlated with increased incidence of tumor recurrence.
Elevated expression of valosin-containing protein (p97) is associated with poor prognosis of prostate cancer.
Genetic prognostic index influences patient outcome for node-positive breast cancer.
In vitro efficacy of a first-generation valosin-containing protein inhibitor (CB-5083) against canine lymphoma.
Inadequate fine-tuning of protein synthesis and failure of amino acid homeostasis following inhibition of the ATPase VCP/p97.
Increased expression of valosin-containing protein (p97) is correlated with disease recurrence in follicular thyroid cancer.
Inhibiting valosin-containing protein suppresses osteosarcoma cell metastasis via AKT/nuclear factor of kappa B signaling pathway in vitro.
Inhibitors of the AAA+ Chaperone p97.
Novel p97/VCP inhibitor induces endoplasmic reticulum stress and apoptosis in both bortezomib-sensitive and -resistant multiple myeloma cells.
p97/VCP is highly expressed in the stem-like cells of breast cancer and controls cancer stemness partly through the unfolded protein response.
Point-of-care test for cervical cancer in LMICs.
Pre-B-cell leukemia transcription factor 1 regulates expression of valosin-containing protein, a gene involved in cancer growth.
Prognostic significance of pre B cell leukemia transcription factor 2 (PBX2) expression in non-small cell lung carcinoma.
Protein kinase A inhibition facilitates the antitumor activity of xanthohumol, a valosin-containing protein inhibitor.
Proteotoxic crisis, the ubiquitin-proteasome system, and cancer therapy.
Synthesis and In Vitro Evaluation of 2-[3-(2-Aminoethyl)-1H-indol-1-yl]-N-benzylquinazolin-4-amine as a Novel p97/VCP Inhibitor Lead Capable of Inducing Apoptosis in Cancer Cells.
Targeting the NPL4 Adaptor of p97/VCP Segregase by Disulfiram as an Emerging Cancer Vulnerability Evokes Replication Stress and DNA Damage while Silencing the ATR Pathway.
Targeting valosin-containing protein enhances the efficacy of radiation therapy in esophageal squamous cell carcinoma.
The p97-UFD1L-NPL4 protein complex mediates cytokine-induced I?B? proteolysis.
Tumor Suppressor RARRES1 Regulates DLG2, PP2A, VCP, EB1, and Ankrd26.
Ubiquitin proteasome system and autophagy associated proteins in human testicular tumors.
Upregulation of valosin-containing protein (VCP) is associated with poor prognosis and promotes tumor progression of orbital B-cell lymphoma.
Valosin-containing protein (p97) and Ki-67 expression is a useful marker in detecting malignant behavior of pancreatic endocrine neoplasms.
Valosin-containing protein (VCP/p97)-expression correlates with prognosis of HPV- negative oropharyngeal squamous cell carcinoma (OSCC).
Valosin-containing protein cleavage by granzyme K accelerates an endoplasmic reticulum stress leading to caspase-independent cytotoxicity of target tumor cells.
Valosin-containing Protein in Psoriasis: A Clinical and Immunohistochemical Study.
Valosin-containing Protein is a Target of 5'-l Fuligocandin?B and Enhances TRAIL Resistance in Cancer Cells.
Nervous System Diseases
Mutational analysis of the VCP gene in Parkinson's disease.
Neuroblastoma
Enterovirus 71 induces neural cell apoptosis and autophagy through promoting ACOX1 downregulation and ROS generation.
Neurodegenerative Diseases
Drosophila Valosin-Containing Protein is required for dendrite pruning through a regulatory role in mRNA metabolism.
Functional ATPase activity of p97/valosin-containing protein (VCP) is required for the quality control of endoplasmic reticulum in neuronally differentiated mammalian PC12 cells.
Increased expression of valosin-containing protein in the skin of patients with amyotrophic lateral sclerosis.
Lysine methylation of VCP by a member of a novel human protein methyltransferase family.
Neuronal remodeling and apoptosis require VCP-dependent degradation of the apoptosis inhibitor DIAP1.
Next Generation Sequencing and ALS: known genes, different phenotyphes.
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies.
Physical and functional interaction between Dorfin and Valosin-containing protein that are colocalized in ubiquitylated inclusions in neurodegenerative disorders.
Rare variants in SQSTM1 and VCP genes and risk of sporadic inclusion body myositis.
The wide-ranging clinical and genetic features in Japanese families with valosin-containing protein proteinopathy.
Valosin-containing protein (VCP) in novel feedback machinery between abnormal protein accumulation and transcriptional suppression.
Osteitis Deformans
A novel exon 2 I27V VCP variant is associated with dissimilar clinical syndromes.
A novel mutation in the VCP gene (G157R) in a German family with inclusion-body myopathy with Paget disease of bone and frontotemporal dementia.
A progressive translational mouse model of human valosin-containing protein disease: the VCP(R155H/+) mouse.
Altered cofactor regulation with disease-associated p97/VCP mutations.
An Italian family with inclusion-body myopathy and frontotemporal dementia due to mutation in the VCP gene.
APOE is a potential modifier gene in an autosomal dominant form of frontotemporal dementia (IBMPFD).
Brain imaging and neuropsychology in late-onset dementia due to a novel mutation (R93C) of valosin-containing protein.
Ceramide contributes to pathogenesis and may be targeted for therapy in VCP inclusion body myopathy.
Characterization of the Asian myopathy patients with VCP mutations.
Clinical heterogeneity in 3 unrelated families linked to VCP p.Arg159His.
Clinical outcome in 19 French and Spanish patients with valosin-containing protein myopathy associated with Paget's disease of bone and frontotemporal dementia.
Early-onset Alzheimers and Cortical Vision Impairment in a Woman With Valosin-containing Protein Disease Associated With 2 APOE [Latin Small Letter Open E]4/APOE [Latin Small Letter Open E]4 Genotype.
Etiologic factors in Paget's disease of bone.
Evaluation of the role of Valosin-containing protein in the pathogenesis of familial and sporadic Paget's disease of bone.
Global Gene Expression Profiling in R155H Knock-In Murine Model of VCP Disease.
Global gene profiling of VCP-associated inclusion body myopathy.
Hereditary inclusion body myopathy-linked p97/VCP mutations in the NH2 domain and the D1 ring modulate p97/VCP ATPase activity and D2 ring conformation.
Identification and characterization of valosin-containing protein (VCP/p97) in untransformed osteoblast-like cells.
Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein.
Inclusion Body Myopathy With Paget Disease of Bone and Frontotemporal Dementia Linked to VCP p.Arg155Cys in a Korean Family.
Late-onset autosomal dominant limb girdle muscular dystrophy and Paget's disease of bone unlinked to the VCP gene locus.
Lipid-enriched diet rescues lethality and slows down progression in a murine model of VCP-associated disease.
Multisystem proteinopathy: Where myopathy and motor neuron disease converge.
Neuronal-specific overexpression of a mutant valosin-containing protein associated with IBMPFD promotes aberrant ubiquitin and TDP-43 accumulation and cognitive dysfunction in transgenic mice.
Novel ubiquitin neuropathology in frontotemporal dementia with valosin-containing protein gene mutations.
Novel valosin-containing protein mutations associated with multisystem proteinopathy.
Novel VCP mutations expand the mutational spectrum of frontotemporal dementia.
Novel VCP mutations in inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia.
Nuclear inclusions mimicking poly(A)-binding protein nuclear 1 inclusions in a case of inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia with a novel mutation in the valosin-containing protein gene.
Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model.
Phenotypic diversity in an international Cure VCP Disease registry.
Radiological features of Paget disease of bone associated with VCP myopathy.
Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice.
SQSTM1 mutations in Han Chinese populations with sporadic amyotrophic lateral sclerosis.
Strumpellin is a novel valosin-containing protein binding partner linking hereditary spastic paraplegia to protein aggregation diseases.
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations.
TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.
Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice.
Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.
Valosin-containing protein (VCP) is a novel IQ motif-containing GTPase activating protein 1 (IQGAP1)-interacting protein.
Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy.
[A case of inclusion body myopathy with Paget's disease of bone and frontotemporal dementia (IBMPFD) showing clinical features of motor neuron disease].
Osteosarcoma
Demethylation-mediated miR-129-5p up-regulation inhibits malignant phenotype of osteogenic osteosarcoma by targeting Homo sapiens valosin-containing protein (VCP).
Identification and characterization of valosin-containing protein (VCP/p97) in untransformed osteoblast-like cells.
Inhibiting valosin-containing protein suppresses osteosarcoma cell metastasis via AKT/nuclear factor of kappa B signaling pathway in vitro.
Knockdown of Aurora-B alters osteosarcoma cell malignant phenotype via decreasing phosphorylation of VCP and NF-?B signaling.
Paget's Disease of Bone: Evidence for Complex Pathogenetic Interactions.
Valosin-containing protein promotes metastasis of osteosarcoma through autophagy induction and anoikis inhibition via the ERK/NF-??/beclin-1 signaling pathway.
Pancreatic Neoplasms
Valosin-Containing Protein Stabilizes Mutant p53 to Promote Pancreatic Cancer Growth.
Paraplegia
Novel spastin (SPG4) mutations in Italian patients with hereditary spastic paraplegia.
Spastin mutations are frequent in sporadic spastic paraparesis and their spectrum is different from that observed in familial cases.
Spastin, a new AAA protein, is altered in the most frequent form of autosomal dominant spastic paraplegia.
SPG7 targets the m-AAA protease complex to process MCU for uniporter assembly, Ca2+ influx, and regulation of mitochondrial permeability transition pore opening.
Parkinson Disease
Enhanced ATPase activities as a primary defect of mutant valosin-containing proteins that cause inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia.
SVIP is a molecular determinant of lysosomal dynamic stability, neurodegeneration and lifespan.
Valosin-containing protein mutation and Parkinson's disease.
Parkinsonian Disorders
Parkinsonian syndrome in familial frontotemporal dementia.
Parkinsonism in a patient with valosin-containing protein gene mutation showing: a case report.
Perinatal Death
Peroxisomal protein PEX13 functions in selective autophagy.
Pituitary Neoplasms
Modified cell cycle status in a mouse model of altered neuronal vulnerability (slow Wallerian degeneration; Wlds).
Polyneuropathies
Variant analysis of PEX11B gene from a family with peroxisome biogenesis disorder 14B by whole exome sequencing.
Pre-Eclampsia
Altered expression of p97/Valosin containing protein and impaired autophagy in preeclamptic human placenta.
Precursor B-Cell Lymphoblastic Leukemia-Lymphoma
Pre-B-cell leukemia transcription factor 1 regulates expression of valosin-containing protein, a gene involved in cancer growth.
Prognostic significance of pre B cell leukemia transcription factor 2 (PBX2) expression in non-small cell lung carcinoma.
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Bortezomib Treatment can Overcome Glucocorticoid Resistance in Childhood B-cell Precursor Acute Lymphoblastic Leukemia Cell Lines.
Prostatic Neoplasms
Chemistry and biology for the small molecules targeting characteristics of cancer cells.
Elevated expression of valosin-containing protein (p97) is associated with poor prognosis of prostate cancer.
Interleukin-6 induced overexpression of valosin-containing protein (VCP)/p97 is associated with androgen-independent prostate cancer (AIPC) progression.
Psoriasis
Valosin-containing Protein in Psoriasis: A Clinical and Immunohistochemical Study.
Pulmonary Disease, Chronic Obstructive
Critical role of proteostasis-imbalance in pathogenesis of COPD and severe emphysema.
Refsum Disease
Phenotype of adult Refsum disease due to a defect in peroxin 7.
Retinal Degeneration
Expression of p97/VCP and ubiquitin during postnatal development of the degenerating rat retina.
Retinal Diseases
Modulation of valosin-containing protein by Kyoto University Substances (KUS) as a novel therapeutic strategy for ischemic neuronal diseases.
Retinitis Pigmentosa
Efficient Ocular Delivery of VCP siRNA via Reverse Magnetofection in RHO P23H Rodent Retina Explants.
Rift Valley Fever
Sorafenib impedes Rift Valley fever virus egress by inhibiting Valosin-containing protein function in the cellular secretory pathway.
Sarcoma
Amyotrophic lateral sclerosis, frontotemporal lobar dementia, and p62: A functional convergence?
Frontotemporal dementia: from mendelian genetics towards genome wide association studies.
Frontotemporal dementia: implications for understanding Alzheimer disease.
Genetics of Alzheimer's Disease and Frontotemporal Dementia.
Parkinsonian syndrome in familial frontotemporal dementia.
Phenotypic Heterogeneity of Monogenic Frontotemporal Dementia.
Seizures
PEX1 deficiency presenting as Leber congenital amaurosis.
Spastic Paraplegia, Hereditary
Hereditary Spastic Paraplegia with a Novel SPAST Mutation Misdiagnosed with Subacute Combined Degeneration.
Strumpellin is a novel valosin-containing protein binding partner linking hereditary spastic paraplegia to protein aggregation diseases.
Squamous Cell Carcinoma of Head and Neck
Valosin-containing protein (VCP/p97)-expression correlates with prognosis of HPV- negative oropharyngeal squamous cell carcinoma (OSCC).
Starvation
The peroxin Pex14p is involved in LC3-dependent degradation of mammalian peroxisomes.
Stomach Diseases
Subcellular and functional proteomic analysis of the cellular responses induced by Helicobacter pylori.
Synucleinopathies
Valosin-containing protein immunoreactivity in tauopathies, synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease.
Tauopathies
Valosin-containing protein immunoreactivity in tauopathies, synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease.
TDP-43 Proteinopathies
ALS and FTLD: two faces of TDP-43 proteinopathy.
Frontotemporal dementias: update on recent developments in molecular genetics and neuropathology.
TDP-43 in neurodegenerative disorders.
Testicular Neoplasms
Ubiquitin proteasome system and autophagy associated proteins in human testicular tumors.
Thyroid Neoplasms
Increased expression of valosin-containing protein (p97) is correlated with disease recurrence in follicular thyroid cancer.
Uterine Cervical Neoplasms
Lon Peptidase 2, Peroxisomal (LONP2) Contributes to Cervical Carcinogenesis via Oxidative Stress.
Werner Syndrome
Werner syndrome protein directly binds to the AAA ATPase p97/VCP in an ATP-dependent fashion.
Wiskott-Aldrich Syndrome
Genetic analysis of VCP and WASH complex genes in a German cohort of sporadic ALS-FTD patients.
Yellow Fever
A Sensitive Yellow Fever Virus Entry Reporter Identifies Valosin-Containing Protein (VCP/p97) as an Essential Host Factor for Flavivirus Uncoating.
Zellweger Syndrome
A cytoplasmic AAA family peroxin, Pex1p, interacts with Pex6p.
Drosophila carrying pex3 or pex16 mutations are models of zellweger syndrome that reflect its symptoms associated with the absence of peroxisomes.
Genetic and clinical aspects of Zellweger spectrum patients with PEX1 mutations.
Human peroxisome assembly factor-2 (PAF-2): a gene responsible for group C peroxisome biogenesis disorder in humans.
Organelle disease: peroxisomal disorders.
Peroxisomal protein PEX13 functions in selective autophagy.
Peroxisome biogenesis and molecular defects in peroxisome assembly disorders.
The membrane biogenesis peroxin Pex16p. Topogenesis and functional roles in peroxisomal membrane assembly.