Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
about
Machado-Joseph Disease: from first descriptions to new perspectives.Deubiquitylating enzymes and diseaseValosin-containing protein (VCP/p97) is an activator of wild-type ataxin-3Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis.Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtinProteotoxic stress increases nuclear localization of ataxin-3Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3An expanded glutamine repeat destabilizes native ataxin-3 structure and mediates formation of parallel beta -fibrils.Therapeutic prospects for spinocerebellar ataxia type 2 and 3.PolyQ-expanded ataxin-3 interacts with full-length ataxin-3 in a polyQ length-dependent manner.Nucleocytoplasmic shuttling activity of ataxin-3.Pathogenesis of inclusion bodies in (CAG)n/Qn-expansion diseases with special reference to the role of tissue transglutaminase and to selective vulnerability.Evaluation of Antisense Oligonucleotides Targeting ATXN3 in SCA3 Mouse ModelsSplice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties.A major role for side-chain polyglutamine hydrogen bonding in irreversible ataxin-3 aggregation.Spinocerebellar ataxia type 1--modeling the pathogenesis of a polyglutamine neurodegenerative disorder in transgenic mice.Genomic structure, promoter activity, and developmental expression of the mouse homologue of the Machado-Joseph disease (MJD) geneThe molecular biology of the autosomal-dominant cerebellar ataxias.Axonal inclusions in spinocerebellar ataxia type 3.Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117.Expression of ataxin-2 in brains from normal individuals and patients with Alzheimer's disease and spinocerebellar ataxia 2.Role of inositol 1,4,5-trisphosphate receptors in pathogenesis of Huntington's disease and spinocerebellar ataxiasHsp104 suppresses polyglutamine-induced degeneration post onset in a drosophila MJD/SCA3 model.A knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript.Allele-specific silencing of dominant disease genes.Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregatesBalancing act: deubiquitinating enzymes in the nervous system.The role of the mammalian DNA end-processing enzyme polynucleotide kinase 3'-phosphatase in spinocerebellar ataxia type 3 pathogenesis.Nonneural nuclear inclusions of androgen receptor protein in spinal and bulbar muscular atrophy.Toward understanding Machado-Joseph diseaseA mutant ataxin-3 fragment results from processing at a site N-terminal to amino acid 190 in brain of Machado-Joseph disease-like transgenic mice.Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation.A polyalanine tract expansion in Arx forms intranuclear inclusions and results in increased cell death.Mouse models of Machado-Joseph disease and other polyglutamine spinocerebellar ataxias.Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 modelsMachado-Joseph disease/spinocerebellar ataxia type 3.Purification of transcripts and metabolites from Drosophila headsCaloric restriction blocks neuropathology and motor deficits in Machado-Joseph disease mouse models through SIRT1 pathway.Fibroblasts of Machado Joseph Disease patients reveal autophagy impairment.
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Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
description
1997 nî lūn-bûn
@nan
1997 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
1997 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
1997年の論文
@ja
1997年論文
@yue
1997年論文
@zh-hant
1997年論文
@zh-hk
1997年論文
@zh-mo
1997年論文
@zh-tw
1997年论文
@wuu
name
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@ast
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@en
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@en-gb
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@nl
type
label
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@ast
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@en
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@en-gb
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@nl
prefLabel
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@ast
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@en
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@en-gb
Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@nl
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P921
P3181
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Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain
@en
P2093
D Gotsdiner
H L Paulson
K H Fischbeck
R N Pittman
P304
P3181
P356
10.1002/ANA.410410408
P407
P577
1997-04-01T00:00:00Z