View source for 3bpt


==Crystal structure of human beta-hydroxyisobutyryl-CoA hydrolase in complex with quercetin==
<StructureSection load='3bpt' size='340' side='right'caption='[[3bpt]], [[Resolution|resolution]] 1.50&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[3bpt]] is a 1 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=3BPT OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3BPT 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]] 1.5&#8491;</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=HIU:(2R)-3-HYDROXY-2-METHYLPROPANOIC+ACID'>HIU</scene>, <scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene>, <scene name='pdbligand=QUE:3,5,7,3,4-PENTAHYDROXYFLAVONE'>QUE</scene></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=3bpt FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3bpt OCA], [https://pdbe.org/3bpt PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3bpt RCSB], [https://www.ebi.ac.uk/pdbsum/3bpt PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3bpt ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/HIBCH_HUMAN HIBCH_HUMAN] Defects in HIBCH are the cause of HIBCH deficiency (HIBCHD) [MIM:[https://omim.org/entry/250620 250620]; also known as deficiency of beta-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. The enzyme defect results in accumulation of methacrylyl-CoA, a highly reactive compound, which readily undergoes addition reactions with free sulfhydryl groups. Affected individuals showed delayed development of motor skills, hypotonia, initial poor feeding, and a deterioration in neurological function during first stages of life.<ref>PMID:17160907</ref> 
== Function ==
[https://www.uniprot.org/uniprot/HIBCH_HUMAN HIBCH_HUMAN] Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.<ref>PMID:8824301</ref> 
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
  <jmolCheckbox>
    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/bp/3bpt_consurf.spt"</scriptWhenChecked>
    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview03.spt</scriptWhenUnchecked>
    <text>to colour the structure by Evolutionary Conservation</text>
  </jmolCheckbox>
</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=3bpt ConSurf].
<div style="clear:both"></div>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Arrowsmith CH]]
[[Category: Edwards AM]]
[[Category: Guo K]]
[[Category: King ONF]]
[[Category: Oppermann U]]
[[Category: Phillips C]]
[[Category: Pike ACW]]
[[Category: Pilka ES]]
[[Category: Weigelt J]]
[[Category: Von Delft F]]