Metabolomics,Unknown,Transcriptomics,Genomics,Proteomics

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Capicua-dependent transcriptional changes in adult mouse cerebellum

ABSTRACT: Analysis of cerebella from Capicua (Cic) mutant mice and wild-type controls at 28 days of age (P28). Spinocerebellar ataxia type 1 (SCA1) is a fatal neurodegenerative disease caused by expansion of a translated CAG repeat in Ataxin-1 (ATXN1). The transcriptional repressor Cic binds directly to Atxn1 and plays a key role in SCA1 pathogenesis. Two isoforms of Cic, long (Cic-L) and short (Cic-S), are transcribed from alternative promoters. Using embryonic stem cells in which the Cic locus was targeted by an insertion of a genetrap cassette between exon 1 of the Cic-L isoform and exon 1 of the Cic-S isoform, we generated mice that carried this allele and backcrossed these onto a Swiss Webster (CD-1) strain for >6 generations. The resulting Cic-L-/- mice completely lack the Cic-L isoform with ~10% of Cic-S remaining. These data were used to compare with previous microarray data to determine the Cic-depedent pathogenic mechanisms in SCA1. Total RNA from cerebella of wild-type (n=4) and Capicua mutant mice (n=4) at 28 days of age was prepared and labeled according the manufacturer's protocols for the Affymetrix Mouse Gene 1.0 ST Array.

ORGANISM(S): Mus musculus

SUBMITTER: Peng Yu 

PROVIDER: E-GEOD-32051 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Publications

Exercise and genetic rescue of SCA1 via the transcriptional repressor Capicua.

Fryer John D JD   Yu Peng P   Kang Hyojin H   Mandel-Brehm Caleigh C   Carter Angela N AN   Crespo-Barreto Juan J   Gao Yan Y   Flora Adriano A   Shaw Chad C   Orr Harry T HT   Zoghbi Huda Y HY  

Science (New York, N.Y.) 20111101 6056

Spinocerebellar ataxia type 1 (SCA1) is a fatal neurodegenerative disease caused by expansion of a translated CAG repeat in Ataxin-1 (ATXN1). To determine the long-term effects of exercise, we implemented a mild exercise regimen in a mouse model of SCA1 and found a considerable improvement in survival accompanied by up-regulation of epidermal growth factor and consequential down-regulation of Capicua, which is an ATXN1 interactor. Offspring of Capicua mutant mice bred to SCA1 mice showed signifi  ...[more]

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