1oa8: Difference between revisions
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'''AXH DOMAIN OF HUMAN SPINOCEREBELLAR ATAXIN-1''' | {{Structure | ||
|PDB= 1oa8 |SIZE=350|CAPTION= <scene name='initialview01'>1oa8</scene>, resolution 1.70Å | |||
|SITE= <scene name='pdbsite=AC1:Na+Binding+Site+For+Chain+D'>AC1</scene> | |||
|LIGAND= <scene name='pdbligand=NA:SODIUM ION'>NA</scene> | |||
|ACTIVITY= | |||
|GENE= | |||
}} | |||
'''AXH DOMAIN OF HUMAN SPINOCEREBELLAR ATAXIN-1''' | |||
==Overview== | ==Overview== | ||
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==About this Structure== | ==About this Structure== | ||
1OA8 is a [ | 1OA8 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=1OA8 OCA]. | ||
==Reference== | ==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:[http:// | 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:[http://www.ncbi.nlm.nih.gov/pubmed/14583607 14583607] | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Single protein]] | [[Category: Single protein]] | ||
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[[Category: rna-binding]] | [[Category: rna-binding]] | ||
''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 13:08:00 2008'' | ||
Revision as of 14:08, 20 March 2008
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| Coordinates: | save as pdb, mmCIF, xml | ||||||
AXH DOMAIN OF HUMAN SPINOCEREBELLAR ATAXIN-1
OverviewOverview
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.
DiseaseDisease
Known diseases associated with this structure: Spinocerebellar ataxia-1 OMIM:[601556]
About this StructureAbout this Structure
1OA8 is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
ReferenceReference
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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