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3bpt
From Proteopedia
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| - | [[Image:3bpt.png|left|200px]] | ||
| - | + | ==Crystal structure of human beta-hydroxyisobutyryl-CoA hydrolase in complex with quercetin== | |
| - | + | <StructureSection load='3bpt' size='340' side='right'caption='[[3bpt]], [[Resolution|resolution]] 1.50Å' scene=''> | |
| - | + | == Structural highlights == | |
| - | + | <table><tr><td colspan='2'>[[3bpt]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. 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='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=QUE:3,5,7,3,4-PENTAHYDROXYFLAVONE'>QUE</scene></td></tr> | |
| - | == | + | <tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr> |
| - | [[3bpt]] is a 1 chain structure with sequence from [ | + | <tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">HIBCH ([https://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr> |
| + | <tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[https://en.wikipedia.org/wiki/3-hydroxyisobutyryl-CoA_hydrolase 3-hydroxyisobutyryl-CoA hydrolase], with EC number [https://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.1.2.4 3.1.2.4] </span></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/initialview01.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: 3-hydroxyisobutyryl-CoA hydrolase]] | [[Category: 3-hydroxyisobutyryl-CoA hydrolase]] | ||
| - | [[Category: | + | [[Category: Human]] |
| - | [[Category: Arrowsmith, C H | + | [[Category: Large Structures]] |
| - | [[Category: Delft, F von | + | [[Category: Arrowsmith, C H]] |
| - | [[Category: Edwards, A M | + | [[Category: Delft, F von]] |
| - | [[Category: Guo, K | + | [[Category: Edwards, A M]] |
| - | [[Category: King, O N.F | + | [[Category: Guo, K]] |
| - | [[Category: Oppermann, U | + | [[Category: King, O N.F]] |
| - | [[Category: Phillips, C | + | [[Category: Oppermann, U]] |
| - | [[Category: Pike, A C.W | + | [[Category: Phillips, C]] |
| - | [[Category: Pilka, E S | + | [[Category: Pike, A C.W]] |
| - | [[Category: | + | [[Category: Pilka, E S]] |
| - | [[Category: Weigelt, J | + | [[Category: Structural genomic]] |
| + | [[Category: Weigelt, J]] | ||
[[Category: Beta-hydroxyisobutyryl acid]] | [[Category: Beta-hydroxyisobutyryl acid]] | ||
[[Category: Branched-chain amino acid catabolism]] | [[Category: Branched-chain amino acid catabolism]] | ||
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[[Category: Quercetin]] | [[Category: Quercetin]] | ||
[[Category: Sgc]] | [[Category: Sgc]] | ||
| - | [[Category: Structural genomics consortium]] | ||
[[Category: Transit peptide]] | [[Category: Transit peptide]] | ||
Current revision
Crystal structure of human beta-hydroxyisobutyryl-CoA hydrolase in complex with quercetin
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Categories: 3-hydroxyisobutyryl-CoA hydrolase | Human | Large Structures | Arrowsmith, C H | Delft, F von | Edwards, A M | Guo, K | King, O N.F | Oppermann, U | Phillips, C | Pike, A C.W | Pilka, E S | Structural genomic | Weigelt, J | Beta-hydroxyisobutyryl acid | Branched-chain amino acid catabolism | Coenzyme some | Disease mutation | Hydrolase | Mitochondrion | Quercetin | Sgc | Transit peptide
