Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation - Wikidata - awgldk - TriplyDB

Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation

Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation

http://www.wikidata.org/entity/Q28284708

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How citation distortions create unfounded authority: analysis of a citation network Valosin-containing protein (VCP/p97) is required for poliovirus replication and is involved in cellular protein secretion pathway in poliovirus infection Imbalances in p97 co-factor interactions in human proteinopathy The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology From neurodevelopment to neurodegeneration: the interaction of neurofibromin and valosin-containing protein/p97 in regulation of dendritic spine formation The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathology Detailed structural insights into the p97-Npl4-Ufd1 interface A novel ATP-dependent conformation in p97 N–D1 fragment revealed by crystal structures of disease-related mutants Altered Intersubunit Communication Is the Molecular Basis for Functional Defects of Pathogenic p97 Mutants Mutations in the Human AAA+ Chaperone p97 and Related Diseases VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD 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 The PUB domain functions as a p97 binding module in human peptide N-glycanase Emerging functions of the VCP/p97 AAA-ATPase in the ubiquitin system The requirement for Cdc48/p97 in nuclear protein quality control degradation depends on the substrate and correlates with substrate insolubility Cell biology. Metabolic control of cell death The p97/VCP ATPase is critical in muscle atrophy and the accelerated degradation of muscle proteins Endolysosomal sorting of ubiquitylated caveolin-1 is regulated by VCP and UBXD1 and impaired by VCP disease mutations VCP mutations causing frontotemporal lobar degeneration disrupt localization of TDP-43 and induce cell death.Int6 and Moe1 interact with Cdc48 to regulate ERAD and proper chromosome segregation The role of the UPS in cystic fibrosis.Ubiquitin-mediated signalling and Paget's disease of bone.Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia Continuum A Mighty "Protein Extractor" of the Cell: Structure and Function of the p97/CDC48 ATPase.Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution The VCP/p97 system at a glance: connecting cellular function to disease pathogenesis.Derlin-1 regulates mutant VCP-linked pathogenesis and endoplasmic reticulum stress-induced apoptosis.Coaggregation of RNA-binding proteins in a model of TDP-43 proteinopathy with selective RGG motif methylation and a role for RRM1 ubiquitination Heteromeric p97/p97R155C complexes induce dominant negative changes in wild-type and autophagy 9-deficient Dictyostelium strains Ongoing developments in sporadic inclusion body myositis The complexities of p97 function in health and disease AAA+ ATPases: achieving diversity of function with conserved machinery.Valosin-containing protein gene mutations: cellular phenotypes relevant to neurodegeneration.A network of RNA and protein interactions in Fronto Temporal Dementia.Targeted sequencing and identification of genetic variants in sporadic inclusion body myositis.Valosin-containing protein and neurofibromin interact to regulate dendritic spine density Valosin containing protein associated fronto-temporal lobar degeneration: clinical presentation, pathologic features and pathogenesis.

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P2860

Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation

http://www.wikidata.org/entity/Q28284708

description

2006 nî lūn-bûn

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2006 թուականի Յունուարին հրատարակուած գիտական յօդուած

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2006年の論文

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Inclusion body myopathy-associ ...... ticulum-associated degradation

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Human Molecular Genetics

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Inclusion body myopathy-associ ...... ticulum-associated degradation

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Alan Pestronk

http://www.w3.org/2001/XMLSchema#string

Conrad C Weihl

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Phyllis I Hanson

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Seema Dalal

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P2860

Valosin-containing protein is a multi-ubiquitin chain-targeting factor required in ubiquitin-proteasome degradation

Evidence for a dominant-negative effect in ACTA1 nemaline myopathy caused by abnormal folding, aggregation and altered polymerization of mutant actin isoforms

A membrane protein required for dislocation of misfolded proteins from the ER

A membrane protein complex mediates retro-translocation from the ER lumen into the cytosol

Involvement of valosin-containing protein, an ATPase Co-purified with IkappaBalpha and 26 S proteasome, in ubiquitin-proteasome-mediated degradation of IkappaBalpha

VCIP135 acts as a deubiquitinating enzyme during p97-p47-mediated reassembly of mitotic Golgi fragments

Ataxin-3 interactions with rad23 and valosin-containing protein and its associations with ubiquitin chains and the proteasome are consistent with a role in ubiquitin-mediated proteolysis

The crystal structure of murine p97/VCP at 3.6A

The AAA ATPase Cdc48/p97 and its partners transport proteins from the ER into the cytosol.

AAA-ATPase p97/Cdc48p, a cytosolic chaperone required for endoplasmic reticulum-associated protein degradation.

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scholarly article

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2866341

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10.1093/HMG/DDI426

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2

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15

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7447069

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16321991

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endoplasmic reticulum