4ndf: Difference between revisions
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{{STRUCTURE_4ndf| PDB=4ndf | SCENE= }} | |||
===Human Aprataxin (Aptx) bound to RNA-DNA, AMP, and Zn - product complex=== | |||
==Disease== | |||
[[http://www.uniprot.org/uniprot/APTX_HUMAN APTX_HUMAN]] Defects in APTX are the cause of ataxia-oculomotor apraxia syndrome (AOA) [MIM:[http://omim.org/entry/208920 208920]]. AOA is an autosomal recessive syndrome characterized by early-onset cerebellar ataxia, oculomotor apraxia, early areflexia and late peripheral neuropathy.<ref>PMID:11586299</ref> <ref>PMID:11586300</ref> <ref>PMID:12196655</ref> <ref>PMID:12629250</ref> <ref>PMID:14506070</ref> <ref>PMID:15852392</ref> <ref>PMID:15699391</ref> | |||
==Function== | |||
[[http://www.uniprot.org/uniprot/APTX_HUMAN APTX_HUMAN]] DNA-binding protein involved in single-strand DNA break repair, double-strand DNA break repair and base excision repair. Resolves abortive DNA ligation intermediates formed either at base excision sites, or when DNA ligases attempt to repair non-ligatable breaks induced by reactive oxygen species. Catalyzes the release of adenylate groups covalently linked to 5'-phosphate termini, resulting in the production of 5'-phosphate termini that can be efficiently rejoined. Also able to hydrolyze adenosine 5'-monophosphoramidate (AMP-NH(2)) and diadenosine tetraphosphate (AppppA), but with lower catalytic activity.<ref>PMID:14755728</ref> <ref>PMID:15044383</ref> <ref>PMID:16547001</ref> <ref>PMID:16964241</ref> <ref>PMID:17276982</ref> | |||
==About this Structure== | |||
[[4ndf]] is a 6 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4NDF OCA]. | |||
==Reference== | |||
<references group="xtra"/><references/> | |||
[[Category: Schellenberg, M J.]] | |||
[[Category: Tumbale, P S.]] | |||
[[Category: Williams, R S.]] | |||
[[Category: 5'-dna end processing]] | |||
[[Category: 5'-dna end recognition]] | |||
[[Category: Dna binding protein-rna-dna complex]] | |||
[[Category: Dna repair]] | |||
[[Category: Histidine triad domain]] | |||
[[Category: Hit domain]] | |||
[[Category: Protein-dna complex]] | |||
[[Category: Zinc finger]] | |||