Metabolomics,Unknown,Transcriptomics,Genomics,Proteomics

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Transcription profiling of ataxin-null (Atxn-null) vs. wild type mice to investigate spinocerebellar ataxia type 1 (SCA1)


ABSTRACT: 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 neurodegeneration or apparent neuropathological abnormalities.We extracted RNA from cerebellum of 5 Atxn1-null mice and 5 WT. Cerebellar gene expression profiles at 15 weeks of age were generated usSCA1 ing Affymetrix MOE430A arrays. Identifying the molecular pathways regulated by Atxn1 can provide insights into the early molecular mechanisms underlying neuronal dysfunction.

ORGANISM(S): Mus musculus

SUBMITTER: Tamas Revesz 

PROVIDER: E-MEXP-886 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Publications

Down-regulation of the dopamine receptor D2 in mice lacking ataxin 1.

Goold Robert R   Hubank Michael M   Hunt Abigail A   Holton Janice J   Menon Rajesh P RP   Revesz Tamas T   Pandolfo Massimo M   Matilla-Dueñas Antoni A  

Human molecular genetics 20070628 17


Ataxin 1 (Atxn1) is a protein of unknown function associated with spinocerebellar ataxia type 1 (SCA1), a neurodegenerative disease of late onset with variable degrees of cerebellar ataxia, ophthalmoplegia and neuropathy. SCA1 is caused by the toxic effects triggered by an expanded polyglutamine (polyQ) within Atxn1 resulting in neurodegeneration in the cerebellum, brain stem and spinocerebellar tracts. To gain insights into Atxn1 function, we have analysed the cerebellar gene expression profile  ...[more]

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