Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado–Joseph disease

Machado–Joseph disease is the most frequently found dominantly-inherited cerebellar ataxia. Over-repetition of a CAG trinucleotide in the MJD1 gene translates into a polyglutamine tract within the ataxin 3 protein, which upon proteolysis may trigger Machado–Joseph disease. We investigated the role o...

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Bibliographic Details
Main Author: Simões, Ana T. (author)
Other Authors: Gonçalves, Nélio (author), Koeppen, Arnulf (author), Déglon, Nicole (author), Kügler, Sebastian (author), Duarte, Carlos Bandeira (author), Almeida, Luís Pereira de (author)
Format: article
Language:eng
Published: 2012
Subjects:
Machado–Joseph disease
Spinocerebellar ataxia type 3
Calpastatin
Cleavage fragment
Proteolysis
Online Access:http://hdl.handle.net/10316/28114
Country:Portugal
Oai:oai:estudogeral.sib.uc.pt:10316/28114

Internet

http://hdl.handle.net/10316/28114

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