2aga

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[[Image:2aga.gif|left|200px]]
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{{STRUCTURE_2aga| PDB=2aga | SCENE= }}
{{STRUCTURE_2aga| PDB=2aga | SCENE= }}
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'''De-ubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain'''
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===De-ubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain===
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==Overview==
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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.
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(as it appears on PubMed at http://www.pubmed.gov), where 16118278 is the PubMed ID number.
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{{ABSTRACT_PUBMED_16118278}}
==About this Structure==
==About this Structure==
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2AGA is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2AGA OCA].
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2AGA is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2AGA OCA].
==Reference==
==Reference==
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[[Category: Uim]]
[[Category: Uim]]
[[Category: Vcp/p97]]
[[Category: Vcp/p97]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 19:00:50 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 29 00:32:17 2008''

Revision as of 21:32, 28 July 2008

Image:2aga.png

Template:STRUCTURE 2aga

De-ubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain

Template:ABSTRACT PUBMED 16118278

About this Structure

2AGA is a Single protein structure of sequence from Homo sapiens. Full experimental 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

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