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Article

Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3).

Human molecular genetics - 1 Feb 2013

Hübener Jeannette, Weber Jonasz Jeremiasz, Richter Claudia, Honold Lisa, Weiss Andreas, Murad Fabronia, Breuer Peter, Wüllner Ullrich, Bellstedt Peter, Paquet-Durand Francois, Takano Jiro, Saido Takaomi C, Riess Olaf, Nguyen Huu Phuc

Abstract excerpt

Spinocerebellar ataxia type 3 (SCA3) is pathologically characterized by the formation of intranuclear aggregates which contain ataxin-3, the mutated protein in SCA3, in a specific subtype of neurons. It has been proposed that ataxin-3 is cleaved by proteolytic enzymes, in particular by calpains and caspases, eventually leading to the formation of aggregates. In our study, we examined the ability of calpains to...

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PMID: 23100324
DOI: 10.1093/hmg/dds449

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