The spinocerebellar ataxia type 1 protein, ataxin-1, has RNA-binding activity that is inversely affected by the length of its polyglutamine tract
about
Boat, an AXH domain protein, suppresses the cytotoxicity of mutant ataxin-1.A Drosophila homolog of the polyglutamine disease gene SCA2 is a dosage-sensitive regulator of actin filament formationPathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1Gene, Stem Cell, and Alternative Therapies for SCA 1Cell biology of spinocerebellar ataxiaThe unstable repeats--three evolving faces of neurological diseasePolyglutamine expansion accelerates the dynamics of ataxin-1 and does not result in aggregate formation.The structure of the AXH domain of spinocerebellar ataxin-1Protein-protein interactions as a strategy towards protein-specific drug design: the example of ataxin-1Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1Phosphorylation of S776 and 14-3-3 binding modulate ataxin-1 interaction with splicing factorsThe insulin-like growth factor pathway is altered in spinocerebellar ataxia type 1 and type 7Bovine proteins containing poly-glutamine repeats are often polymorphic and enriched for components of transcriptional regulatory complexes.Regulation of RNA splicing by the methylation-dependent transcriptional repressor methyl-CpG binding protein 2.FOX-2 dependent splicing of ataxin-2 transcript is affected by ataxin-1 overexpressionStabilization and Degradation Mechanisms of Cytoplasmic Ataxin-1.Regulation of microRNA expression by HMGA1 proteins.Partial loss of Tip60 slows mid-stage neurodegeneration in a spinocerebellar ataxia type 1 (SCA1) mouse model.HMGB1 facilitates repair of mitochondrial DNA damage and extends the lifespan of mutant ataxin-1 knock-in mice.Abnormalities in the climbing fiber-Purkinje cell circuitry contribute to neuronal dysfunction in ATXN1[82Q] mice14-3-3 Binding to ataxin-1(ATXN1) regulates its dephosphorylation at Ser-776 and transport to the nucleus.Functional genomic screen and network analysis reveal novel modifiers of tauopathy dissociated from tau phosphorylation.Pathways to motor incoordination: the inherited ataxias.Scaffold function of long non-coding RNA HOTAIR in protein ubiquitinationAmyloidogenesis of natively unfolded proteins.Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias.Kaleidoscopic protein-protein interactions in the life and death of ataxin-1: new strategies against protein aggregation.Nuclear ataxias.Nucleolar aggresomes as counterparts of cytoplasmic aggresomes in proteotoxic stress. Proteasome inhibitors induce nuclear ribonucleoprotein inclusions that accumulate several key factors of neurodegenerative diseases and cancer.Role of metal ions in aggregation of intrinsically disordered proteins in neurodegenerative diseases.Modulation of Molecular Chaperones in Huntington's Disease and Other Polyglutamine Disorders.Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways.Translating cerebellar Purkinje neuron physiology to progress in dominantly inherited ataxia.Ataxin-1 regulates the cerebellar bioenergetics proteome through the GSK3β-mTOR pathway which is altered in Spinocerebellar ataxia type 1 (SCA1).Proteasome inhibitors induce nucleolar aggregation of proteasome target proteins and polyadenylated RNA by altering ubiquitin availability.SUMOylation of the polyglutamine repeat protein, ataxin-1, is dependent on a functional nuclear localization signal.Gene expression profiling in ataxin-3 expressing cell lines reveals distinct effects of normal and mutant ataxin-3.Phosphorylation of ATXN1 at Ser776 in the cerebellum.Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases.Dynamics of CAG repeat loci revealed by the analysis of their variability.
P2860
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P2860
The spinocerebellar ataxia type 1 protein, ataxin-1, has RNA-binding activity that is inversely affected by the length of its polyglutamine tract
description
2001 nî lūn-bûn
@nan
2001 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2001 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2001年の論文
@ja
2001年論文
@yue
2001年論文
@zh-hant
2001年論文
@zh-hk
2001年論文
@zh-mo
2001年論文
@zh-tw
2001年论文
@wuu
name
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@ast
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@en
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@en-gb
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@nl
type
label
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@ast
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@en
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@en-gb
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@nl
prefLabel
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@ast
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@en
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@en-gb
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@nl
P2093
P921
P3181
P356
P1476
The spinocerebellar ataxia typ ...... gth of its polyglutamine tract
@en
P2093
P3181
P356
10.1093/HMG/10.1.25
P407
P50
P577
2001-01-01T00:00:00Z