Transcription profiling of mouse model for spinocerebellar ataxia 1 vs, wild type littermates sampled at 4 and 11 weeks

ABSTRACT: A number of human neurodegenerative diseases result from the expansion of a glutamine repeat within the disease-causing protein. Spinocerebellar ataxia type1 is one such disease, caused by expansion of a polyglutamine tract in the novel protein ataxin-1. To faithfully model SCA1 in the mouse, we generated knock-in mice carrying 154 CAG repeats in the mouse Sca1 locus. These mice reproduced many aspects of the human disease. Despite ubiquitous expression of the mutant protein, they developed slowly progressive selective neurodegeneration , which is most distinct in Purkinje cells and spinal cord. Alterations in gene expression have been proposed to be involved in the pathogenesis of polyglutamine disease including SCA1, but the changes of gene expression in an authentic disease model have

ORGANISM(S): Mus musculus

SUBMITTER: Elizabeth Salomon 

PROVIDER: E-GEOD-2867 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress