1oa8
From Proteopedia
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| - | [[Image:1oa8.jpg|left|200px]]<br /><applet load="1oa8" size=" | + | [[Image:1oa8.jpg|left|200px]]<br /><applet load="1oa8" size="350" color="white" frame="true" align="right" spinBox="true" |
caption="1oa8, resolution 1.70Å" /> | caption="1oa8, resolution 1.70Å" /> | ||
'''AXH DOMAIN OF HUMAN SPINOCEREBELLAR ATAXIN-1'''<br /> | '''AXH DOMAIN OF HUMAN SPINOCEREBELLAR ATAXIN-1'''<br /> | ||
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==About this Structure== | ==About this Structure== | ||
| - | 1OA8 is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with NA as [http://en.wikipedia.org/wiki/ligand ligand]. Known structural/functional Site: <scene name='pdbsite=AC1:Na Binding Site For Chain D'>AC1</scene>. Full crystallographic information is available from [http:// | + | 1OA8 is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with <scene name='pdbligand=NA:'>NA</scene> as [http://en.wikipedia.org/wiki/ligand ligand]. Known structural/functional Site: <scene name='pdbsite=AC1:Na+Binding+Site+For+Chain+D'>AC1</scene>. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1OA8 OCA]. |
==Reference== | ==Reference== | ||
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[[Category: rna-binding]] | [[Category: rna-binding]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Feb 3 09:55:29 2008'' |
Revision as of 07:55, 3 February 2008
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AXH DOMAIN OF HUMAN SPINOCEREBELLAR ATAXIN-1
Contents |
Overview
Spinocerebellar ataxia type 1 is a late-onset neurodegenerative disease, caused by the expansion of a CAG triplet repeat in the SCA1 gene. This, results in the lengthening of a polyglutamine tract in the gene product, ataxin-1. This produces a toxic gain of function that results in specific, neuronal death. A region in ataxin-1, the AXH domain, exhibits significant, sequence similarity to the transcription factor HBP1. This region of the, protein has been implicated in RNA binding and self-association. We have, determined the crystal structure of the AXH domain of ataxin-1. The AXH, domain is dimeric and contains an OB-fold, a structural motif found in, many oligonucleotide-binding proteins, supporting its proposed role in RNA, binding. By structure comparison with other proteins that contain an, OB-fold, a putative RNA-binding site has been identified. We also, identified a cluster of charged surface residues that are well conserved, among AXH domains. These residues may constitute a second ligand-binding, surface, suggesting that all AXH domains interact with a common yet, unidentified partner.
Disease
Known diseases associated with this structure: Spinocerebellar ataxia-1 OMIM:[601556]
About this Structure
1OA8 is a Single protein structure of sequence from Homo sapiens with as ligand. Known structural/functional Site: . Full crystallographic information is available from OCA.
Reference
The structure of the AXH domain of spinocerebellar ataxin-1., Chen YW, Allen MD, Veprintsev DB, Lowe J, Bycroft M, J Biol Chem. 2004 Jan 30;279(5):3758-65. Epub 2003 Oct 28. PMID:14583607
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