Article
Polyglutamine-expanded ataxin-7 upregulates Bax expression by activating p53 in cerebellar and inferior olivary neurons.
Experimental neurology - 1 Aug 2010
Wang Hung-Li, Chou An-Hsun, Lin An-Chi, Chen Si-Ying, Weng Yi-Hshin, Yeh Tu-Hsueh
Abstract excerpt
Spinocerebellar ataxia type 7 (SCA7) is an autosomal dominant neurodegenerative disease caused by polyglutamine-expanded ataxin-7. Prominent SCA7 neurodegeneration is found in the cerebellum and inferior olivary nucleus. Mutant polyglutamine ataxin-7 activated mitochondrial apoptotic pathway and induced apoptotic death of cerebellar and inferior olivary neurons by upregulating mRNA expression of proapoptotic Bax....
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