Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo
about
ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeatsCloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeatsCDK5 protects from caspase-induced Ataxin-3 cleavage and neurodegenerationThe DNAJB6 and DNAJB8 protein chaperones prevent intracellular aggregation of polyglutamine peptidesInteraction between neuronal intranuclear inclusions and promyelocytic leukemia protein nuclear and coiled bodies in CAG repeat diseasesJapanese families with autosomal dominant pure cerebellar ataxia map to chromosome 19p13.1-p13.2 and are strongly associated with mild CAG expansions in the spinocerebellar ataxia type 6 gene in chromosome 19p13.1Satellog: a database for the identification and prioritization of satellite repeats in disease association studies.Mouse models of polyglutamine diseases: review and data table. Part IPathogenic mechanisms and therapeutic strategies in spinobulbar muscular atrophyAtaxin-2 regulates RGS8 translation in a new BAC-SCA2 transgenic mouse modelGenome-wide screen for modifiers of ataxin-3 neurodegeneration in DrosophilaSilencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic miceFunctional ATPase activity of p97/valosin-containing protein (VCP) is required for the quality control of endoplasmic reticulum in neuronally differentiated mammalian PC12 cellsDentatorubral pallidoluysian atrophy (DRPLA) protein is cleaved by caspase-3 during apoptosisAutosomal dominant cerebellar ataxia: phenotypic differences in genetically defined subtypes?Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tractMolecular cloning of brain-specific GD1alpha synthase (ST6GalNAc V) containing CAG/Glutamine repeatsHuntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansionLentivector-mediated rescue from cerebellar ataxia in a mouse model of spinocerebellar ataxia.Neuron-specific proteotoxicity of mutant ataxin-3 in C. elegans: rescue by the DAF-16 and HSF-1 pathwaysPathogenesis of inclusion bodies in (CAG)n/Qn-expansion diseases with special reference to the role of tissue transglutaminase and to selective vulnerability.Intracellular inclusions, pathological markers in diseases caused by expanded polyglutamine tracts?Nuclear aggregation of polyglutamine-expanded ataxin-3: fragments escape the cytoplasmic quality control.Polyglutamine pathogenesis.Androgen receptor mutation in Kennedy's diseaseCAG-polyglutamine-repeat mutations: independence from gene context.Long tract of untranslated CAG repeats is deleterious in transgenic micePast, present and future therapeutics for cerebellar ataxias.What transgenic mice tell us about neurodegenerative disease.Animal models of Kennedy disease.Caspases and neurodegeneration: on the cutting edge of new therapeutic approaches.Toward an understanding of polyglutamine neurodegeneration.Genomic structure, promoter activity, and developmental expression of the mouse homologue of the Machado-Joseph disease (MJD) geneValosin-containing protein (VCP) in novel feedback machinery between abnormal protein accumulation and transcriptional suppressionThe molecular biology of the autosomal-dominant cerebellar ataxias.Protein aggregation and pathogenesis of Huntington's disease: mechanisms and correlations.Molecular clearance of ataxin-3 is regulated by a mammalian E4Interaction of expanded polyglutamine stretches with nuclear transcription factors leads to aberrant transcriptional regulation in polyglutamine diseases.A pathogenic mechanism in Huntington's disease involves small CAG-repeated RNAs with neurotoxic activity.Advances in the genetics of progressive myoclonus epilepsy.
P2860
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P2860
Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo
description
1996 nî lūn-bûn
@nan
1996 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
1996 թվականի հունիսին հրատարակված գիտական հոդված
@hy
1996年の論文
@ja
1996年論文
@yue
1996年論文
@zh-hant
1996年論文
@zh-hk
1996年論文
@zh-mo
1996年論文
@zh-tw
1996年论文
@wuu
name
Expanded polyglutamine in the ...... ell death in vitro and in vivo
@ast
Expanded polyglutamine in the ...... ell death in vitro and in vivo
@en
Expanded polyglutamine in the ...... ell death in vitro and in vivo
@nl
type
label
Expanded polyglutamine in the ...... ell death in vitro and in vivo
@ast
Expanded polyglutamine in the ...... ell death in vitro and in vivo
@en
Expanded polyglutamine in the ...... ell death in vitro and in vivo
@nl
prefLabel
Expanded polyglutamine in the ...... ell death in vitro and in vivo
@ast
Expanded polyglutamine in the ...... ell death in vitro and in vivo
@en
Expanded polyglutamine in the ...... ell death in vitro and in vivo
@nl
P2093
P3181
P356
P1433
P1476
Expanded polyglutamine in the ...... ell death in vitro and in vivo
@en
P2093
P2888
P304
P3181
P356
10.1038/NG0696-196
P407
P577
1996-06-01T00:00:00Z