Article
Suppression of calbindin-D28k expression exacerbates SCA1 phenotype in a disease mouse model.
Cerebellum (London, England) - 1 Sept 2012
Vig Parminder J S, Wei Jinrong, Shao Qingmei, Lopez Maripar E, Halperin Rebecca, Gerber Jill
Abstract excerpt
Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurological disorder caused by the expansion of a polyglutamine tract in the mutant protein ataxin-1. The cerebellar Purkinje cells (PCs) are the major targets of mutant ataxin-1. The mechanism of PC death in SCA1 is not known; however, previous work indicates that downregulation of specific proteins involved in calcium homeostasis and signaling by...
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