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Article

Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro.

Human molecular genetics - 1 Apr 1999

Chai Y, Koppenhafer S L, Shoesmith S J, Perez M K, Paulson H L

Abstract excerpt

Spinocerebellar ataxia type 3, also known as Machado-Joseph disease (SCA3/MJD), is one of at least eight inherited neurodegenerative diseases caused by expansion of a polyglutamine tract in the disease protein. Here we present two lines of evidence implicating the ubiquitin-proteasome pathway in SCA3/MJD pathogenesis. First, studies of both human disease tissue and in vitro models showed redistribution of the 26S...

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PMID: 10072437
DOI: 10.1093/hmg/8.4.673

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