Article
Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2.
Human molecular genetics - 15 Jan 2013
Hansen Stephen T, Meera Pratap, Otis Thomas S, Pulst Stefan M
Abstract excerpt
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominantly inherited disorder, which is caused by a pathological expansion of a polyglutamine (polyQ) tract in the coding region of the ATXN2 gene. Like other ataxias, SCA2 most overtly affects Purkinje cells (PCs) in the cerebellum. Using a transgenic mouse model expressing a full-length ATXN2(Q127)-complementary DNA under control of the Pcp2 promoter (a...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
