Article
[Mechanisms to control degradation of polyglutamine-containing protein].
Rinsho shinkeigaku = Clinical neurology - 1 Nov 2003
Nakayama Kei-Ichi
Abstract excerpt
Machado-Joseph disease (MJD)/Spinocerebellar ataxia type 3 (SCA3) is neurodegenerative disease which is caused by polyglutamine expansion in a responsible gene product, MJD1/Ataxin3. MJD1 has now been shown to undergo ubiquitylation and degradation by proteasome-dependent pathway. MJD1 with expanded polyglutamine tract was more resistant to degradation than normal MJD1. We established an in vitro system of...
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