4cdo

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==Crystal structure of PQBP1 bound to spliceosomal U5-15kD==
==Crystal structure of PQBP1 bound to spliceosomal U5-15kD==
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<StructureSection load='4cdo' size='340' side='right' caption='[[4cdo]], [[Resolution|resolution]] 2.50&Aring;' scene=''>
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<StructureSection load='4cdo' size='340' side='right'caption='[[4cdo]], [[Resolution|resolution]] 2.50&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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[[4cdo]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4CDO OCA]. <br>
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<table><tr><td colspan='2'>[[4cdo]] is a 2 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=4CDO OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4CDO FirstGlance]. <br>
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<b>Activity:</b> <span class='plainlinks'>[http://en.wikipedia.org/wiki/Glucokinase Glucokinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.1.2 2.7.1.2] </span><br>
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</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>
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<b>Resources:</b> <span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4cdo FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4cdo OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4cdo RCSB], [http://www.ebi.ac.uk/pdbsum/4cdo PDBsum]</span><br>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=4cdo FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4cdo OCA], [https://pdbe.org/4cdo PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4cdo RCSB], [https://www.ebi.ac.uk/pdbsum/4cdo PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4cdo ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/PQBP1_HUMAN PQBP1_HUMAN] X-linked intellectual deficit, Sutherland-Haan type;X-linked intellectual deficit, Golabi-Ito-Hall type;X-linked intellectual deficit, Porteous type;Hamel cerebro-palato-cardiac syndrome. The disease is caused by mutations affecting the gene represented in this entry.
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== Function ==
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[https://www.uniprot.org/uniprot/PQBP1_HUMAN PQBP1_HUMAN] May suppress the ability of POU3F2 to transactivate the DRD1 gene in a POU3F2 dependent manner. Can activate transcription directly or via association with the transcription machinery. May be involved in ATXN1 mutant-induced cell death. The interaction with ATXN1 mutant reduces levels of phosphorylated RNA polymerase II large subunit.<ref>PMID:10332029</ref> <ref>PMID:10198427</ref> <ref>PMID:12062018</ref> [https://www.uniprot.org/uniprot/TXN4A_HUMAN TXN4A_HUMAN] Essential role in pre-mRNA splicing.
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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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.
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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>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
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<div class="pdbe-citations 4cdo" style="background-color:#fffaf0;"></div>
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==See Also==
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*[[U5-15kD|U5-15kD]]
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Kojima, R.]]
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[[Category: Homo sapiens]]
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[[Category: Mizuguchi, M.]]
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[[Category: Large Structures]]
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[[Category: Nabeshima, Y.]]
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[[Category: Kojima R]]
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[[Category: Obita, T.]]
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[[Category: Mizuguchi M]]
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[[Category: Okazawa, H.]]
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[[Category: Nabeshima Y]]
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[[Category: Serita, T.]]
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[[Category: Obita T]]
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[[Category: Cell cycle]]
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[[Category: Okazawa H]]
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[[Category: U5-52kd]]
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[[Category: Serita T]]

Current revision

Crystal structure of PQBP1 bound to spliceosomal U5-15kD

PDB ID 4cdo

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