1oa8

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{{STRUCTURE_1oa8| PDB=1oa8 | SCENE= }}
{{STRUCTURE_1oa8| PDB=1oa8 | SCENE= }}
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'''AXH DOMAIN OF HUMAN SPINOCEREBELLAR ATAXIN-1'''
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===AXH DOMAIN OF HUMAN SPINOCEREBELLAR ATAXIN-1===
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==Overview==
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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.
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(as it appears on PubMed at http://www.pubmed.gov), where 14583607 is the PubMed ID number.
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{{ABSTRACT_PUBMED_14583607}}
==About this Structure==
==About this Structure==
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[[Category: Hmg]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 03:35:18 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Jul 28 06:07:46 2008''

Revision as of 03:07, 28 July 2008

Image:1oa8.png

Template:STRUCTURE 1oa8

AXH DOMAIN OF HUMAN SPINOCEREBELLAR ATAXIN-1

Template:ABSTRACT PUBMED 14583607

About this Structure

1OA8 is a Single protein structure of sequence from Homo sapiens. 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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