2aga: Difference between revisions
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'''De-ubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain''' | '''De-ubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain''' | ||
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[[Category: Polo, S.]] | [[Category: Polo, S.]] | ||
[[Category: Senic-Matuglia, F.]] | [[Category: Senic-Matuglia, F.]] | ||
[[Category: | [[Category: Ataxia]] | ||
[[Category: | [[Category: Polyglutamine]] | ||
[[Category: | [[Category: Ubiquitin]] | ||
[[Category: | [[Category: Uim]] | ||
[[Category: | [[Category: Vcp/p97]] | ||
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 19:00:50 2008'' | |||
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Revision as of 16:00, 3 May 2008
De-ubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain
Overview
Spinocerebellar ataxia type 3 is a human neurodegenerative disease resulting from polyglutamine tract expansion. The affected protein, ataxin-3, which contains an N-terminal Josephin domain followed by tandem ubiquitin (Ub)-interacting motifs (UIMs) and a polyglutamine stretch, has been implicated in the function of the Ub proteasome system. NMR-based structural analysis has now revealed that the Josephin domain binds Ub and has a papain-like fold that is reminiscent of that of other deubiquitinases, despite primary sequence divergence but consistent with its deubiqutinating activity. Mutation of the catalytic Cys enhances the stability of a complex between ataxin-3 and polyubiquitinated proteins. This effect depends on the integrity of the UIM region, suggesting that the UIMs are bound to the substrate polyubiquitin during catalysis. We propose that ataxin-3 functions as a polyubiquitin chain-editing enzyme.
About this Structure
2AGA is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Deubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain., Mao Y, Senic-Matuglia F, Di Fiore PP, Polo S, Hodsdon ME, De Camilli P, Proc Natl Acad Sci U S A. 2005 Sep 6;102(36):12700-5. Epub 2005 Aug 23. PMID:16118278 Page seeded by OCA on Sat May 3 19:00:50 2008
