This old version of Proteopedia is provided for student assignments while the new version is undergoing repairs. Content and edits done in this old version of Proteopedia after March 1, 2026 will eventually be lost when it is retired in about June of 2026.
Apply for new accounts at the new Proteopedia. Your logins will work in both the old and new versions.
1oa8
From Proteopedia
| Line 1: | Line 1: | ||
| - | [[Image:1oa8.jpg|left|200px]] | + | [[Image:1oa8.jpg|left|200px]] |
| - | + | ||
| - | '''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== | ||
| Line 10: | Line 19: | ||
==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]] | ||
| Line 25: | Line 34: | ||
[[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 11:08, 20 March 2008
| |||||||
| , resolution 1.70Å | |||||||
|---|---|---|---|---|---|---|---|
| Sites: | |||||||
| Ligands: | |||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||
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. 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
Page seeded by OCA on Thu Mar 20 13:08:00 2008
