Transcription profiling of mouse knock in mutant model for spinocerebellar ataxia type 1 and type 7 - symptomatic Sca1154Q/2Q and Sca7266Q/5Q cerebellum

ABSTRACT: Comparative analysis of cerebellar gene expression changes occurring in Sca1154Q/2Q and Sca7266Q/5Q knock-in mice; Polyglutamine diseases are inherited neurodegenerative disorders caused by expansion of CAG repeats encoding a glutamine tract in the disease-causing proteins. There are nine disorders each having distinct features but also clinical and pathological similarities. In particular, spinocerebellar ataxia type 1 and type 7 (SCA1 and SCA7) patients manifest cerebellar ataxia with degeneration of Purkinje cells. To determine whether the disorders share molecular pathogenic events, we studied two mouse models of SCA1 and SCA7 that express the glutamine-expanded protein from the respective endogenous loci. We found common transcriptional changes, with down-regulation of Insulin-lik

ORGANISM(S): Mus musculus

SUBMITTER: Chad Shaw 

PROVIDER: E-GEOD-9914 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress