Ubiquitin ligase Hrd1 enhances the degradation and suppresses the toxicity of polyglutamine-expanded huntingtin
about
SVIP induces localization of p97/VCP to the plasma and lysosomal membranes and regulates autophagyAn E3 ubiquitin ligase, Synoviolin, is involved in the degradation of immature nicastrin, and regulates the production of amyloid beta-proteinEffects of overexpression of huntingtin proteins on mitochondrial integrity.Huntingtin interacts with the cue domain of gp78 and inhibits gp78 binding to ubiquitin and p97/VCP.Ubiquitin ligase SYVN1/HRD1 facilitates degradation of the SERPINA1 Z variant/α-1-antitrypsin Z variant via SQSTM1/p62-dependent selective autophagy.The E3 ubiquitin ligases Hrd1 and gp78 bind to and promote cholera toxin retro-translocation.Stringent requirement for HRD1, SEL1L, and OS-9/XTP3-B for disposal of ERAD-LS substrates.Proteostasis in striatal cells and selective neurodegeneration in Huntington's disease.Serine residues in the cytosolic tail of the T-cell antigen receptor alpha-chain mediate ubiquitination and endoplasmic reticulum-associated degradation of the unassembled protein.The endoplasmic reticulum (ER)-associated degradation system regulates aggregation and degradation of mutant neuroserpin.Effects of oxidative stress on the solubility of HRD1, a ubiquitin ligase implicated in Alzheimer's diseaseImportin beta interacts with the endoplasmic reticulum-associated degradation machinery and promotes ubiquitination and degradation of mutant alpha1-antitrypsinDecreases in valosin-containing protein result in increased levels of tau phosphorylated at Ser262/356.Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3.Familial prion protein mutants inhibit Hrd1-mediated retrotranslocation of misfolded proteins by depleting misfolded protein sensor BiP.Impaired protein aggregate handling and clearance underlie the pathogenesis of p97/VCP-associated disease.The de-ubiquitinating enzyme ataxin-3 does not modulate disease progression in a knock-in mouse model of Huntington disease.Cross-functional E3 ligases Parkin and C-terminus Hsp70-interacting protein in neurodegenerative disorders.E3 ubiquitin ligases in protein quality control mechanism.Signaling pathways from the endoplasmic reticulum and their roles in diseaseUbiquitin pathways in neurodegenerative disease.Mahogunin Ring Finger-1 (MGRN1), a Multifaceted Ubiquitin Ligase: Recent Unraveling of Neurobiological Mechanisms.A critical role of Hrd1 in the regulation of optineurin degradation and aggresome formation.UPS Activation in the Battle Against Aging and Aggregation-Related Diseases: An Extended Review.The evolving role of ubiquitin modification in endoplasmic reticulum-associated degradation.The Ubiquitin Receptor ADRM1 Modulates HAP40-Induced Proteasome Activity.HRD1 levels increased by zonisamide prevented cell death and caspase-3 activation caused by endoplasmic reticulum stress in SH-SY5Y cells.Proteostasis of Huntingtin in Health and DiseaseDetection of ubiquitinated huntingtin species in intracellular aggregates.Evolutionary divergence of valosin-containing protein/cell division cycle protein 48 binding interactions among endoplasmic reticulum-associated degradation proteins.Tumor necrosis factor receptor-associated factor 6 (TRAF6) associates with huntingtin protein and promotes its atypical ubiquitination to enhance aggregate formation.Deficiency of Ube3a in Huntington's disease mice brain increases aggregate load and accelerates disease pathology.Roles for the VCP co-factors Npl4 and Ufd1 in neuronal function in Drosophila melanogaster.Azadiradione Restores Protein Quality Control and Ameliorates the Disease Pathogenesis in a Mouse Model of Huntington's Disease.The ubiquitin-proteasome system functionally links neuronal Tomosyn-1 to dendritic morphology.Proteostasis in Huntington's disease: disease mechanisms and therapeutic opportunities.
P2860
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P2860
Ubiquitin ligase Hrd1 enhances the degradation and suppresses the toxicity of polyglutamine-expanded huntingtin
description
2007 nî lūn-bûn
@nan
2007 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
name
Ubiquitin ligase Hrd1 enhances ...... yglutamine-expanded huntingtin
@ast
Ubiquitin ligase Hrd1 enhances ...... yglutamine-expanded huntingtin
@en
Ubiquitin ligase Hrd1 enhances ...... yglutamine-expanded huntingtin
@nl
type
label
Ubiquitin ligase Hrd1 enhances ...... yglutamine-expanded huntingtin
@ast
Ubiquitin ligase Hrd1 enhances ...... yglutamine-expanded huntingtin
@en
Ubiquitin ligase Hrd1 enhances ...... yglutamine-expanded huntingtin
@nl
prefLabel
Ubiquitin ligase Hrd1 enhances ...... yglutamine-expanded huntingtin
@ast
Ubiquitin ligase Hrd1 enhances ...... yglutamine-expanded huntingtin
@en
Ubiquitin ligase Hrd1 enhances ...... yglutamine-expanded huntingtin
@nl
P2093
P1476
Ubiquitin ligase Hrd1 enhances ...... yglutamine-expanded huntingtin
@en
P2093
David C Rubinsztein
Mervyn J Monteiro
Petek Ballar
Shengyun Fang
Shouqing Luo
Xiaoyan Zhong
Yuxian Shen
P304
P356
10.1016/J.YEXCR.2006.10.031
P407
P577
2007-02-01T00:00:00Z