Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1
about
Identification of human proteins that modify misfolding and proteotoxicity of pathogenic ataxin-1The human proteins MBD5 and MBD6 associate with heterochromatin but they do not bind methylated DNAAtaxin-1 and Brother of ataxin-1 are components of the Notch signalling pathwayPumilio1 haploinsufficiency leads to SCA1-like neurodegeneration by increasing wild-type Ataxin1 levelsPrion-like domains as epigenetic regulators, scaffolds for subcellular organization, and drivers of neurodegenerative diseaseStructural studies of CNG repeatsBeyond the glutamine expansion: influence of posttranslational modifications of ataxin-1 in the pathogenesis of spinocerebellar ataxia type 1Current understanding of the role of microRNAs in spinocerebellar ataxiasLarge Polyglutamine Repeats Cause Muscle Degeneration in SCA17 Mice.Atomic resolution structure of CAG RNA repeats: structural insights and implications for the trinucleotide repeat expansion diseasesSelf-Assembly and Conformational Heterogeneity of the AXH Domain of Ataxin-1: An Unusual Example of a Chameleon FoldProtein-protein interactions as a strategy towards protein-specific drug design: the example of ataxin-1Phosphorylation of S776 and 14-3-3 binding modulate ataxin-1 interaction with splicing factorsUbe2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIPConformational fluctuations of the AXH monomer of Ataxin-1.'Costa da Morte' ataxia is spinocerebellar ataxia 36: clinical and genetic characterization.RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1Loss of function of ATXN1 increases amyloid beta-protein levels by potentiating beta-secretase processing of beta-amyloid precursor protein.Inhibition of colony-stimulating factor 1 receptor early in disease ameliorates motor deficits in SCA1 mice.The histone deacetylase HDAC3 is essential for Purkinje cell function, potentially complicating the use of HDAC inhibitors in SCA1An out-of-frame overlapping reading frame in the ataxin-1 coding sequence encodes a novel ataxin-1 interacting proteinChemical shift assignment of the ataxin-1 AXH domain in complex with a CIC ligand peptide.Polyglutamine repeats are associated to specific sequence biases that are conserved among eukaryotes.Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis.FOX-2 dependent splicing of ataxin-2 transcript is affected by ataxin-1 overexpressionBroad distribution of ataxin 1 silencing in rhesus cerebella for spinocerebellar ataxia type 1 therapy.The vasculome of the mouse brain.Ataxin-2 intermediate-length polyglutamine expansions in European ALS patientsThe SAGA histone deubiquitinase module controls yeast replicative lifespan via Sir2 interaction.Distinct neurochemical profiles of spinocerebellar ataxias 1, 2, 6, and cerebellar multiple system atrophy.Role of inositol 1,4,5-trisphosphate receptors in pathogenesis of Huntington's disease and spinocerebellar ataxiasAssessing recovery from neurodegeneration in spinocerebellar ataxia 1: Comparison of in vivo magnetic resonance spectroscopy with motor testing, gene expression and histology.Energy landscapes of functional proteins are inherently risky.Exercise and genetic rescue of SCA1 via the transcriptional repressor Capicua.Retrotransposon activation contributes to fragile X premutation rCGG-mediated neurodegenerationDeranged calcium signaling in Purkinje cells and pathogenesis in spinocerebellar ataxia 2 (SCA2) and other ataxiasA Coding Variant of ANO10, Affecting Volume Regulation of Macrophages, Is Associated with Borrelia Seropositivity.A native interactor scaffolds and stabilizes toxic ATAXIN-1 oligomers in SCA1.RNA-binding protein misregulation in microsatellite expansion disordersToward understanding Machado-Joseph disease
P2860
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P2860
Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1
description
2009 nî lūn-bûn
@nan
2009 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2009 թվականի մարտին հրատարակված գիտական հոդված
@hy
2009年の論文
@ja
2009年論文
@yue
2009年論文
@zh-hant
2009年論文
@zh-hk
2009年論文
@zh-mo
2009年論文
@zh-tw
2009年论文
@wuu
name
Pathogenic mechanisms of a pol ...... spinocerebellar ataxia type 1
@ast
Pathogenic mechanisms of a pol ...... spinocerebellar ataxia type 1
@en
Pathogenic mechanisms of a pol ...... spinocerebellar ataxia type 1
@nl
type
label
Pathogenic mechanisms of a pol ...... spinocerebellar ataxia type 1
@ast
Pathogenic mechanisms of a pol ...... spinocerebellar ataxia type 1
@en
Pathogenic mechanisms of a pol ...... spinocerebellar ataxia type 1
@nl
prefLabel
Pathogenic mechanisms of a pol ...... spinocerebellar ataxia type 1
@ast
Pathogenic mechanisms of a pol ...... spinocerebellar ataxia type 1
@en
Pathogenic mechanisms of a pol ...... spinocerebellar ataxia type 1
@nl
P2860
P921
P3181
P356
P1476
Pathogenic mechanisms of a pol ...... spinocerebellar ataxia type 1
@en
P2860
P304
P3181
P356
10.1074/JBC.R800041200
P407
P577
2009-03-20T00:00:00Z