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A protein aggregation model of human ataxin-1(Q82) was generated in human mesenchymal cells using the Sleeping Beauty transposon system. These cells inducibly express the pathogenic protein that causes SCA1 disease. Here, we describe the transcriptional changes occurring in cells which contain diffe...
ORGANISM(S): Homo sapiens 
To identify protein partners of ataxin-1 in neuronal cells under control or stress conditions, we use complementary proteomics strategies of proximity-dependent biotin identification (BioID) and affinity purification (via GFP-trap pulldown) in Neuro-2a cells expressing epitope-tagged forms of ataxin...
ORGANISM(S): Mus musculus (Mouse) 
2018-10-18 | PXD010352 | Pride
Ataxin 1 (Atxn1) is a protein of unknown function associated with cerebellar neurodegeneration in spinocerebellar ataxia type 1 (SCA1). SCA1 is caused by an expanded polyglutamine within Atxn1 by gain-of-function mechanisms. Lack of Atxn1 in mice triggers motor deficits in the absence of neurodegene...
ORGANISM(S): Mus musculus 
Spinocerebellar ataxia type-1 is caused by an abnormally expanded polyglutamine (polyQ) tract in ataxin-1 protein. These expansions are responsible for protein misfolding and self-assembly into intranuclear inclusion bodies (IIBs) that are linked to neuronal death. Here, we describe a nuclear protei...
ORGANISM(S): Homo sapiens (Human) 
2020-02-18 | PXD012709 | Pride
RNA-seq of human mesenchymal stem cells inducibly overexpressing human ataxin-1(Q82)
Human Ataxin-2 (ATXN2) gene locus variants have been associated with obesity, diabetes mellitus type 1 and hypertension in genome-wide association studies, while mouse studies showed the knock-out of Atxn2 to lead to obesity, insulin resistance and dyslipidemia. Intriguingly, the deficiency of ATXN2...
ORGANISM(S): Mus Musculus (ncbitaxon:10090) 
2020-02-07 | MSV000084933 | MassIVE
Mutant ataxin-1 (Atxn1), which causes spinocerebellar ataxia type 1 (SCA1), binds to and impairs the function of high mobility group box 1 (HMGB1), a critical nuclear protein that regulates DNA architectural changes essential for DNA damage repair and transcription. In this study, we established tha...
ORGANISM(S): Mus musculus 
Genomics
HEK-293T cells expressing mutant ataxin-1
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