2aga
From Proteopedia
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| - | [[Image:2aga.gif|left|200px]] | + | [[Image:2aga.gif|left|200px]] |
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| - | '''De-ubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain''' | + | {{Structure |
| + | |PDB= 2aga |SIZE=350|CAPTION= <scene name='initialview01'>2aga</scene> | ||
| + | |SITE= | ||
| + | |LIGAND= | ||
| + | |ACTIVITY= | ||
| + | |GENE= ATXN3, ATX3, MJD, MJD1, SCA3 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens]) | ||
| + | }} | ||
| + | |||
| + | '''De-ubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain''' | ||
| + | |||
==Overview== | ==Overview== | ||
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==About this Structure== | ==About this Structure== | ||
| - | 2AGA is a [ | + | 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]. |
==Reference== | ==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:[http:// | + | 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:[http://www.ncbi.nlm.nih.gov/pubmed/16118278 16118278] |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Single protein]] | [[Category: Single protein]] | ||
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[[Category: vcp/p97]] | [[Category: vcp/p97]] | ||
| - | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 15:49:23 2008'' |
Revision as of 13:49, 20 March 2008
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| Gene: | ATXN3, ATX3, MJD, MJD1, SCA3 (Homo sapiens) | ||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||
De-ubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain
Contents |
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.
Disease
Known diseases associated with this structure: Machado-Joseph disease OMIM:[607047]
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 Thu Mar 20 15:49:23 2008
Categories: Homo sapiens | Single protein | Camilli, P De. | Fiore, P Di. | Hodsdon, M E. | Mao, Y. | Polo, S. | Senic-Matuglia, F. | Ataxia | Polyglutamine | Ubiquitin | Uim | Vcp/p97
