Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence
about
Valosin-containing protein (VCP/p97) is an activator of wild-type ataxin-3CDK5 protects from caspase-induced Ataxin-3 cleavage and neurodegenerationCell biology of spinocerebellar ataxiaMouse models of polyglutamine diseases: review and data table. Part ISilencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic miceProteotoxic stress increases nuclear localization of ataxin-3Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3Decreased survival and hepato-renal pathology in mice with C-terminally truncated GP73 (GOLPH2)Therapeutic prospects for spinocerebellar ataxia type 2 and 3.Trinucleotide repeats: a structural perspectiveLentivector-mediated rescue from cerebellar ataxia in a mouse model of spinocerebellar ataxia.Knockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6Neuron-specific proteotoxicity of mutant ataxin-3 in C. elegans: rescue by the DAF-16 and HSF-1 pathwaysNucleocytoplasmic shuttling activity of ataxin-3.Increased mitophagy in the skeletal muscle of spinal and bulbar muscular atrophy patientsNuclear aggregation of polyglutamine-expanded ataxin-3: fragments escape the cytoplasmic quality control.Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties.Axonal inclusions in spinocerebellar ataxia type 3.RNA interference mitigates motor and neuropathological deficits in a cerebellar mouse model of Machado-Joseph diseaseBrain pathology of spinocerebellar ataxias.Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia Type 3 miceA knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript.Balancing act: deubiquitinating enzymes in the nervous system.DnaJ-1 and karyopherin α3 suppress degeneration in a new Drosophila model of Spinocerebellar Ataxia Type 6.Toward understanding Machado-Joseph diseaseMachado-Joseph disease/spinocerebellar ataxia type 3.Fibroblasts of Machado Joseph Disease patients reveal autophagy impairment.Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteinsCytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy.Emerging pathogenic pathways in the spinocerebellar ataxias.Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease.Genetically engineered mouse models of the trinucleotide-repeat spinocerebellar ataxias.Autophagy and polyglutamine diseases.RNA therapy for polyglutamine neurodegenerative diseases.Mouse models of spinocerebellar ataxia type 3 (Machado-Joseph disease).Ataxin-3 protein and RNA toxicity in spinocerebellar ataxia type 3: current insights and emerging therapeutic strategies.From pathways to targets: understanding the mechanisms behind polyglutamine disease.Pathology and function of nuclear amyloid. Protein homeostasis matters.Unravelling Endogenous MicroRNA System Dysfunction as a New Pathophysiological Mechanism in Machado-Joseph Disease.Modulation of Molecular Chaperones in Huntington's Disease and Other Polyglutamine Disorders.
P2860
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P2860
Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence
description
2007 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
artículu científicu espublizáu en 2007
@ast
im Juli 2007 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2007/07/11)
@sk
vědecký článek publikovaný v roce 2007
@cs
wetenschappelijk artikel (gepubliceerd op 2007/07/11)
@nl
наукова стаття, опублікована в липні 2007
@uk
مقالة علمية (نشرت في 11-7-2007)
@ar
name
Nuclear localization of ataxin ...... toms in SCA3: in vivo evidence
@ast
Nuclear localization of ataxin ...... toms in SCA3: in vivo evidence
@en
Nuclear localization of ataxin ...... toms in SCA3: in vivo evidence
@nl
type
label
Nuclear localization of ataxin ...... toms in SCA3: in vivo evidence
@ast
Nuclear localization of ataxin ...... toms in SCA3: in vivo evidence
@en
Nuclear localization of ataxin ...... toms in SCA3: in vivo evidence
@nl
prefLabel
Nuclear localization of ataxin ...... toms in SCA3: in vivo evidence
@ast
Nuclear localization of ataxin ...... toms in SCA3: in vivo evidence
@en
Nuclear localization of ataxin ...... toms in SCA3: in vivo evidence
@nl
P2093
P50
P3181
P1476
Nuclear localization of ataxin ...... toms in SCA3: in vivo evidence
@en
P2093
Franco Laccone
Hartwig Wolburg
Jeannette Hübener
Johannes Wilbertz
Karina Häbig
Lukas Rüttiger
Marlies Knipper
Michael Bonin
Sven Poths
P304
P3181
P356
10.1523/JNEUROSCI.4540-06.2007
P407
P577
2007-07-01T00:00:00Z