Article
Mutant Ataxin-1 Inhibits Neural Progenitor Cell Proliferation in SCA1.
Cerebellum (London, England) - 1 Apr 2017
Cvetanovic Marija, Hu Yuan-Shih, Opal Puneet
Abstract excerpt
Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited neurodegenerative disease caused by the expansion of a polyglutamine (Q) repeat tract in the protein ataxin-1 (ATXN1). Beginning as a cerebellar ataxic disorder, SCA1 progresses to involve the cerebral cortex, hippocampus, and brainstem. Using SCA1 knock-in mice that mirror the complexity of the human disease, we report a significant decrease in the...
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