Polyglutamine-expanded ataxin-3 activates mitochondrial apoptotic pathway by upregulating Bax and downregulating Bcl-xL

Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant neurodegenerative disease caused by polyglutamine-expanded ataxin-3. In the present study, we expressed disease-causing mutant ataxin-3-Q79 in neuronal cultures of cerebellum, striatum and substantia nigra by using recombinant adenoviruse...

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Main Authors: An-Hsun Chou, Tu-Hsueh Yeh, Yu-Li Kuo, Yu-Cheng Kao, Mei-Jie Jou, Chia-Yu Hsu, Shu-Ru Tsai, Akira Kakizuka, Hung-Li Wang
Format: Article
Language:English
Published: Elsevier 2006-02-01
Series:Neurobiology of Disease
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996105002123