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- W2128573741 abstract "Machado–Joseph disease (MJD), the most common dominantly inherited ataxia worldwide, is caused by a polyglutamine (polyQ) expansion in the deubiquitinating (DUB) enzyme ataxin-3. Interestingly, MJD can present clinically with features of Parkinsonism. In this study, we identify parkin, an E3 ubiquitin-ligase responsible for a common familial form of Parkinson's disease, as a novel ataxin-3 binding partner. The interaction between ataxin-3 and parkin is direct, involves multiple domains and is greatly enhanced by parkin self-ubiquitination. Moreover, ataxin-3 deubiquitinates parkin directly in vitro and in cells. Compared with wild-type ataxin-3, MJD-linked polyQ-expanded mutant ataxin-3 is more active, possibly owing to its greater efficiency at DUB K27- and K29-linked Ub conjugates on parkin. Remarkably, mutant but not wild-type ataxin-3 promotes the clearance of parkin via the autophagy pathway. The finding is consistent with the reduction in parkin levels observed in the brains of transgenic mice over-expressing polyQ-expanded but not wild-type ataxin-3, raising the intriguing possibility that increased turnover of parkin may contribute to the pathogenesis of MJD and help explain some of its parkinsonian features." @default.
- W2128573741 created "2016-06-24" @default.
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- W2128573741 date "2010-10-11" @default.
- W2128573741 modified "2023-10-07" @default.
- W2128573741 title "The Machado–Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability" @default.
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- W2128573741 doi "https://doi.org/10.1093/hmg/ddq452" @default.
- W2128573741 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3005906" @default.
- W2128573741 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/20940148" @default.
- W2128573741 hasPublicationYear "2010" @default.
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