In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis.
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Phenotype and frequency of STUB1 mutations: next-generation screenings in Caucasian ataxia and spastic paraplegia cohortsUbiquitin-specific protease 25 functions in Endoplasmic Reticulum-associated degradationA Decade of Boon or Burden: What Has the CHIP Ever Done for Cellular Protein Quality Control Mechanism Implicated in Neurodegeneration and Aging?Co-chaperone HSJ1a dually regulates the proteasomal degradation of ataxin-3Therapeutic prospects for spinocerebellar ataxia type 2 and 3.Ataxia and hypogonadism caused by the loss of ubiquitin ligase activity of the U box protein CHIPNeuron-specific proteotoxicity of mutant ataxin-3 in C. elegans: rescue by the DAF-16 and HSF-1 pathwaysAncient origin of animal U-box ubiquitin ligases.Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties.Heat shock protein 70 (hsp70) as an emerging drug target.Assessing the contribution of heterogeneous distributions of oligomers to aggregation mechanisms of polyglutamine peptidesUbiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23A knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript.E2 conjugating enzyme selectivity and requirements for function of the E3 ubiquitin ligase CHIP.Molecular and functional characterization of the only known hemiascomycete ortholog of the carboxyl terminus of Hsc70-interacting protein CHIP in the yeast Yarrowia lipolytica.Cytoplasmic Ubiquitin-Specific Protease 19 (USP19) Modulates Aggregation of Polyglutamine-Expanded Ataxin-3 and Huntingtin through the HSP90 Chaperone.Machado-Joseph disease/spinocerebellar ataxia type 3.Emerging evidence of coding mutations in the ubiquitin-proteasome system associated with cerebellar ataxiasStressing Out Hsp90 in Neurotoxic Proteinopathies.Aggregation formation in the polyglutamine diseases: protection at a cost?Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy.Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease.Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease.Modifiers and mechanisms of multi-system polyglutamine neurodegenerative disorders: lessons from fly models.Genetically engineered mouse models of the trinucleotide-repeat spinocerebellar ataxias.Cross-functional E3 ligases Parkin and C-terminus Hsp70-interacting protein in neurodegenerative disorders.Mouse models of spinocerebellar ataxia type 3 (Machado-Joseph disease).E3 ubiquitin ligases in protein quality control mechanism.Ubiquitin pathways in neurodegenerative disease.From pathways to targets: understanding the mechanisms behind polyglutamine disease.Discovery of Therapeutic Approaches for Polyglutamine Diseases: A Summary of Recent Efforts.Modulation of Molecular Chaperones in Huntington's Disease and Other Polyglutamine Disorders.Interaction of the polyglutamine protein ataxin-3 with Rad23 regulates toxicity in Drosophila models of Spinocerebellar Ataxia Type 3.Repair or destruction-an intimate liaison between ubiquitin ligases and molecular chaperones in proteostasisMouse ataxin-3 functional knock-out model.Normal ATXN3 Allele but Not CHIP Polymorphisms Modulates Age at Onset in Machado-Joseph Disease.Chaperone-dependent Neurodegeneration: A Molecular Perspective on Therapeutic Intervention.Excitation-induced ataxin-3 aggregation in neurons from patients with Machado-Joseph disease.Pathogenic mutation in the ALS/FTD gene, CCNF, causes elevated Lys48-linked ubiquitylation and defective autophagy.Comparison of spinocerebellar ataxia type 3 mouse models identifies early gain-of-function, cell-autonomous transcriptional changes in oligodendrocytes.
P2860
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P2860
In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年学术文章
@wuu
2008年学术文章
@zh-cn
2008年学术文章
@zh-hans
2008年学术文章
@zh-my
2008年学术文章
@zh-sg
2008年學術文章
@yue
2008年學術文章
@zh
2008年學術文章
@zh-hant
name
In vivo suppression of polyglu ...... egation model of pathogenesis.
@en
In vivo suppression of polyglu ...... s of Hsp70-interacting protein
@nl
type
label
In vivo suppression of polyglu ...... egation model of pathogenesis.
@en
In vivo suppression of polyglu ...... s of Hsp70-interacting protein
@nl
prefLabel
In vivo suppression of polyglu ...... egation model of pathogenesis.
@en
In vivo suppression of polyglu ...... s of Hsp70-interacting protein
@nl
P2093
P2860
P1476
In vivo suppression of polyglu ...... regation model of pathogenesis
@en
P2093
Henry L Paulson
Tina M Knutson
Veronica F Colomer Gould
P2860
P304
P356
10.1016/J.NBD.2008.10.016
P577
2008-11-08T00:00:00Z