Impaired protein aggregate handling and clearance underlie the pathogenesis of p97/VCP-associated disease.
about
Valosin-containing protein (VCP/p97) is required for poliovirus replication and is involved in cellular protein secretion pathway in poliovirus infectionImbalances in p97 co-factor interactions in human proteinopathySpatial regulation of UBXD8 and p97/VCP controls ATGL-mediated lipid droplet turnoverThe UBX protein SAKS1 negatively regulates endoplasmic reticulum-associated degradation and p97-dependent degradationEukaryotic stress granules are cleared by autophagy and Cdc48/VCP functionDirect and/or Indirect Roles for SUMO in Modulating Alpha-Synuclein ToxicityCardiac-Restricted Expression of VCP/TER94 RNAi or Disease Alleles Perturbs Drosophila Heart Structure and Impairs Function.The ubiquitin-selective chaperone Cdc48/p97 associates with Ubx3 to modulate monoubiquitylation of histone H2B.VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFDHereditary inclusion body myopathy-linked p97/VCP mutations in the NH2 domain and the D1 ring modulate p97/VCP ATPase activity and D2 ring conformationThe requirement for Cdc48/p97 in nuclear protein quality control degradation depends on the substrate and correlates with substrate insolubilityEndolysosomal sorting of ubiquitylated caveolin-1 is regulated by VCP and UBXD1 and impaired by VCP disease mutationsKnockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6Chromatin retention of DNA damage sensors DDB2 and XPC through loss of p97 segregase causes genotoxicity.Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP diseaseRole of Cigarette Smoke-Induced Aggresome Formation in Chronic Obstructive Pulmonary Disease-Emphysema PathogenesisPathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD modelInclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagySpecific inhibition of p97/VCP ATPase and kinetic analysis demonstrate interaction between D1 and D2 ATPase domainsValosin-containing protein (VCP) in novel feedback machinery between abnormal protein accumulation and transcriptional suppressionAn aggregation sensing reporter identifies leflunomide and teriflunomide as polyglutamine aggregate inhibitors.Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43.The VCP/p97 system at a glance: connecting cellular function to disease pathogenesis.Interaction of SQSTM1 with the motor protein dynein--SQSTM1 is required for normal dynein function and trafficking.The ubiquitin-selective segregase VCP/p97 orchestrates the response to DNA double-strand breaks.p97-containing complexes in proliferation control and cancer: emerging culprits or guilt by association?Heteromeric p97/p97R155C complexes induce dominant negative changes in wild-type and autophagy 9-deficient Dictyostelium strainsGranulostasis: Protein Quality Control of RNP Granules.VCP phosphorylation-dependent interaction partners prevent apoptosis in Helicobacter pylori-infected gastric epithelial cellsThe complexities of p97 function in health and diseaseType 2 transglutaminase in Huntington's disease: a double-edged sword with clinical potential.Valosin-containing protein gene mutations: cellular phenotypes relevant to neurodegeneration.Quantitative cell-based protein degradation assays to identify and classify drugs that target the ubiquitin-proteasome system.Exercise training reverses skeletal muscle atrophy in an experimental model of VCP disease.Proteotoxic crisis, the ubiquitin-proteasome system, and cancer therapy.Rapamycin and chloroquine: the in vitro and in vivo effects of autophagy-modifying drugs show promising results in valosin containing protein multisystem proteinopathy.Valosin containing protein associated fronto-temporal lobar degeneration: clinical presentation, pathologic features and pathogenesis.Global gene profiling of VCP-associated inclusion body myopathy.Protective role of cell division cycle 48 (CDC48) protein against neurodegeneration via ubiquitin-proteasome system dysfunction during zebrafish developmentImpaired autophagosome clearance contributes to cardiomyocyte death in ischemia/reperfusion injury.
P2860
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P2860
Impaired protein aggregate handling and clearance underlie the pathogenesis of p97/VCP-associated disease.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
2008年论文
@zh
2008年论文
@zh-cn
name
Impaired protein aggregate han ...... of p97/VCP-associated disease.
@en
type
label
Impaired protein aggregate han ...... of p97/VCP-associated disease.
@en
prefLabel
Impaired protein aggregate han ...... of p97/VCP-associated disease.
@en
P2093
P2860
P356
P1476
Impaired protein aggregate han ...... of p97/VCP-associated disease.
@en
P2093
Conrad C Weihl
Jeong-Sun Ju
Phyllis I Hanson
Sara E Miller
P2860
P304
30289-30299
P356
10.1074/JBC.M805517200
P407
P577
2008-08-20T00:00:00Z