View source for 4bws


==Crystal structure of the heterotrimer of PQBP1, U5-15kD and U5-52kD.==
<StructureSection load='4bws' size='340' side='right'caption='[[4bws]], [[Resolution|resolution]] 2.50&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[4bws]] is a 6 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4BWS OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4BWS FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.5&#8491;</td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=4bws FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4bws OCA], [https://pdbe.org/4bws PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4bws RCSB], [https://www.ebi.ac.uk/pdbsum/4bws PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4bws ProSAT]</span></td></tr>
</table>
== Function ==
[https://www.uniprot.org/uniprot/TXN4A_HUMAN TXN4A_HUMAN] Essential role in pre-mRNA splicing.
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
A loss-of-function of polyglutamine tract-binding protein 1 (PQBP1) induced by frameshift mutations is believed to cause X-linked mental retardation. However, the mechanism by which structural changes in PQBP1 lead to mental retardation is unknown. Here we present the crystal structure of a C-terminal fragment of PQBP1 in complex with the spliceosomal protein U5-15kD. The U5-15kD hydrophobic groove recognizes a YxxPxxVL motif in PQBP1, and mutations within this motif cause a loss-of-function phenotype of PQBP1 in vitro. The YxxPxxVL motif is absent in all PQBP1 frameshift mutants seen in cases of mental retardation. These results suggest a mechanism by which the loss of the YxxPxxVL motif could lead to the functional defects seen in this type of mental retardation.

Mutations in the PQBP1 gene prevent its interaction with the spliceosomal protein U5-15kD.,Mizuguchi M, Obita T, Serita T, Kojima R, Nabeshima Y, Okazawa H Nat Commun. 2014 Apr 30;5:3822. doi: 10.1038/ncomms4822. PMID:24781215<ref>PMID:24781215</ref>

From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
<div class="pdbe-citations 4bws" style="background-color:#fffaf0;"></div>

==See Also==
*[[U5-15kD|U5-15kD]]
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Kojima R]]
[[Category: Mizuguchi M]]
[[Category: Morimoto T]]
[[Category: Nabeshima Y]]
[[Category: Obita T]]
[[Category: Okazawa H]]
[[Category: Serita T]]