Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3.
about
Valosin-containing protein (VCP/p97) is an activator of wild-type ataxin-3Regulation of retrotranslocation by p97-associated deubiquitinating enzyme ataxin-3Crystal structure of a Josephin-ubiquitin complex: evolutionary restraints on ataxin-3 deubiquitinating activityUbiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3Efficient protection and isolation of ubiquitylated proteins using tandem ubiquitin-binding entitiesUbiquitin-specific protease 25 functions in Endoplasmic Reticulum-associated degradationAtaxin-3 is a multivalent ligand for the parkin Ubl domainThe ubiquitin proteasome system in neuropathologyStructural Basis for Ubiquitin Recognition by a Novel Domain from Human Phospholipase A2-activating ProteinTrinucleotide repeats: a structural perspectiveHistone acetylation, acetyltransferases, and ataxia--alteration of histone acetylation and chromatin dynamics is implicated in the pathogenesis of polyglutamine-expansion disordersPolyglutamine genes interact to modulate the severity and progression of neurodegeneration in Drosophila.Allele-specific RNA silencing of mutant ataxin-3 mediates neuroprotection in a rat model of Machado-Joseph disease.Ataxin-3 promotes genome integrity by stabilizing Chk1.Ataxin-3 plays a role in mouse myogenic differentiation through regulation of integrin subunit levelsStructural transformation of the tandem ubiquitin-interacting motifs in ataxin-3 and their cooperative interactions with ubiquitin chains.Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties.Axonal inclusions in spinocerebellar ataxia type 3.Trehalose improves human fibroblast deficits in a new CHIP-mutation related ataxia.Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117.Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23Dominant negative effect of polyglutamine expansion perturbs normal function of ataxin-3 in neuronal cells.Toward understanding Machado-Joseph diseaseAtaxin-3 regulates aggresome formation of copper-zinc superoxide dismutase (SOD1) by editing K63-linked polyubiquitin chains.Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 modelsMachado-Joseph disease/spinocerebellar ataxia type 3.JosD1, a membrane-targeted deubiquitinating enzyme, is activated by ubiquitination and regulates membrane dynamics, cell motility, and endocytosisStrategies for the identification of novel inhibitors of deubiquitinating enzymes.Aggregation of polyglutamine-expanded ataxin-3 sequesters its specific interacting partners into inclusions: implication in a loss-of-function pathology.Regulation of proteolysis by human deubiquitinating enzymes.Ataxin-3 protein and RNA toxicity in spinocerebellar ataxia type 3: current insights and emerging therapeutic strategies.Polyglutamine (PolyQ) diseases: genetics to treatments.The UBA-UIM domains of the USP25 regulate the enzyme ubiquitination state and modulate substrate recognition.The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains.Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease.Cellular turnover of the polyglutamine disease protein ataxin-3 is regulated by its catalytic activity.p45, an ATPase subunit of the 19S proteasome, targets the polyglutamine disease protein ataxin-3 to the proteasome.Ataxin-3 consolidates the MDC1-dependent DNA double-strand break response by counteracting the SUMO-targeted ubiquitin ligase RNF4.Deregulation of autophagy in postmortem brains of Machado-Joseph disease patients.Identification of a novel site of interaction between ataxin-3 and the amyloid aggregation inhibitor polyglutamine binding peptide 1.
P2860
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P2860
Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3.
description
2005 nî lūn-bûn
@nan
2005年の論文
@ja
2005年学术文章
@wuu
2005年学术文章
@zh-cn
2005年学术文章
@zh-hans
2005年学术文章
@zh-my
2005年学术文章
@zh-sg
2005年學術文章
@yue
2005年學術文章
@zh
2005年學術文章
@zh-hant
name
Defining the role of ubiquitin ...... ine disease protein, ataxin-3.
@en
type
label
Defining the role of ubiquitin ...... ine disease protein, ataxin-3.
@en
prefLabel
Defining the role of ubiquitin ...... ine disease protein, ataxin-3.
@en
P2093
P2860
P356
P1476
Defining the role of ubiquitin ...... ine disease protein, ataxin-3.
@en
P2093
Ginger L Marrs
Henry L Paulson
Hsiang Wen
Sarah J Shoesmith Berke
Yaohui Chai
P2860
P304
32026-32034
P356
10.1074/JBC.M506084200
P407
P577
2005-07-21T00:00:00Z