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A mutant ataxin-3 fragment results from processing at a site N-terminal to amino acid 190 in brain of Machado-Joseph disease-like transgenic mice.

Neurobiology of disease - 1 Sept 2007

Colomer Gould Veronica F, Goti Daniel, Pearce Donna, Gonzalez Guillermo A, Gao Hong, Bermudez de Leon Mario, Jenkins Nancy A, Copeland Neal G, Ross Christopher A, Brown Dale R

Abstract excerpt

Machado-Joseph disease also called spinocerebellar ataxia type 3 (MJD/SCA3) is a hereditary and neurodegenerative movement disorder caused by ataxin-3 with a polyglutamine expansion (mutant ataxin-3). Neuronal loss in MJD/SCA3 is associated with a mutant ataxin-3 toxic fragment. Defining mutant ataxin-3 proteolytic site(s) could facilitate the identification of the corresponding enzyme(s). Previously, we reported...

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Identifiers

PMID: 17632007
DOI: 10.1016/j.nbd.2007.06.005
PMCID: PMC2040168

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