1uw4: Difference between revisions
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<StructureSection load='1uw4' size='340' side='right' caption='[[1uw4]], [[Resolution|resolution]] 1.95Å' scene=''> | <StructureSection load='1uw4' size='340' side='right' caption='[[1uw4]], [[Resolution|resolution]] 1.95Å' scene=''> | ||
== Structural highlights == | == Structural highlights == | ||
<table><tr><td colspan='2'>[[1uw4]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/ | <table><tr><td colspan='2'>[[1uw4]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1UW4 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1UW4 FirstGlance]. <br> | ||
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=BME:BETA-MERCAPTOETHANOL'>BME</scene></td></tr> | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=BME:BETA-MERCAPTOETHANOL'>BME</scene></td></tr> | ||
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1uw4 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1uw4 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=1uw4 RCSB], [http://www.ebi.ac.uk/pdbsum/1uw4 PDBsum]</span></td></tr> | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1uw4 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1uw4 OCA], [http://pdbe.org/1uw4 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=1uw4 RCSB], [http://www.ebi.ac.uk/pdbsum/1uw4 PDBsum]</span></td></tr> | ||
</table> | </table> | ||
== Disease == | |||
[[http://www.uniprot.org/uniprot/REN3B_HUMAN REN3B_HUMAN]] FG syndrome;X-linked intellectual disability with marfanoid habitus;X-linked non-syndromic intellectual disability. The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:17704778</ref> | |||
== Function == | |||
[[http://www.uniprot.org/uniprot/REN3B_HUMAN REN3B_HUMAN]] Involved in nonsense-mediated decay (NMD) of mRNAs containing premature stop codons by associating with the nuclear exon junction complex (EJC) and serving as link between the EJC core and NMD machinery. Recruits UPF2 at the cytoplasmic side of the nuclear envelope and the subsequent formation of an UPF1-UPF2-UPF3 surveillance complex (including UPF1 bound to release factors at the stalled ribosome) is believed to activate NMD. In cooperation with UPF2 stimulates both ATPase and RNA helicase activities of UPF1. Binds spliced mRNA upstream of exon-exon junctions. In vitro, stimulates translation; the function is independent of association with UPF2 and components of the EJC core.<ref>PMID:11163187</ref> <ref>PMID:12718880</ref> <ref>PMID:16209946</ref> <ref>PMID:16601204</ref> <ref>PMID:18066079</ref> [[http://www.uniprot.org/uniprot/RENT2_HUMAN RENT2_HUMAN]] Involved in nonsense-mediated decay (NMD) of mRNAs containing premature stop codons by associating with the nuclear exon junction complex (EJC). Recruited by UPF3B associated with the EJC core at the cytoplasmic side of the nuclear envelope and the subsequent formation of an UPF1-UPF2-UPF3 surveillance complex (including UPF1 bound to release factors at the stalled ribosome) is believed to activate NMD. In cooperation with UPF3B stimulates both ATPase and RNA helicase activities of UPF1. Binds spliced mRNA.<ref>PMID:11163187</ref> <ref>PMID:16209946</ref> <ref>PMID:18066079</ref> | |||
== Evolutionary Conservation == | == Evolutionary Conservation == | ||
[[Image:Consurf_key_small.gif|200px|right]] | [[Image:Consurf_key_small.gif|200px|right]] | ||
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | ||
</div> | </div> | ||
<div class="pdbe-citations 1uw4" style="background-color:#fffaf0;"></div> | |||
== References == | == References == | ||
<references/> | <references/> | ||
__TOC__ | __TOC__ | ||
</StructureSection> | </StructureSection> | ||
[[Category: | [[Category: Human]] | ||
[[Category: Cusack, S]] | [[Category: Cusack, S]] | ||
[[Category: Izaurralde, E]] | [[Category: Izaurralde, E]] | ||