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

Neurobiology of disease - 1 Feb 2006

Chou An-Hsun, Yeh Tu-Hsueh, Kuo Yu-Li, Kao Yu-Cheng, Jou Mei-Jie, Hsu Chia-Yu, Tsai Shu-Ru, Kakizuka Akira, Wang Hung-Li

Abstract excerpt

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 adenoviruses. Subsequently, SCA3 cellular model was used to investigate the molecular mechanism by which ataxin-3-Q79 causes...

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PMID: 16112867
DOI: 10.1016/j.nbd.2005.07.011

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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