3bpt

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== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[3bpt]] is a 1 chain structure with sequence from [http://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 [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3BPT FirstGlance]. <br>
<table><tr><td colspan='2'>[[3bpt]] is a 1 chain structure with sequence from [http://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 [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3BPT FirstGlance]. <br>
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</td></tr><tr><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=HIU:(2R)-3-HYDROXY-2-METHYLPROPANOIC+ACID'>HIU</scene>, <scene name='pdbligand=QUE:3,5,7,3,4-PENTAHYDROXYFLAVONE'>QUE</scene><br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><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>
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<tr><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>
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<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>
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<tr><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">HIBCH ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">HIBCH ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
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<tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/3-hydroxyisobutyryl-CoA_hydrolase 3-hydroxyisobutyryl-CoA hydrolase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.1.2.4 3.1.2.4] </span></td></tr>
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<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/3-hydroxyisobutyryl-CoA_hydrolase 3-hydroxyisobutyryl-CoA hydrolase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.1.2.4 3.1.2.4] </span></td></tr>
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<tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3bpt FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3bpt OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3bpt RCSB], [http://www.ebi.ac.uk/pdbsum/3bpt PDBsum]</span></td></tr>
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<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=3bpt FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3bpt OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3bpt RCSB], [http://www.ebi.ac.uk/pdbsum/3bpt PDBsum]</span></td></tr>
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<table>
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</table>
== Disease ==
== Disease ==
[[http://www.uniprot.org/uniprot/HIBCH_HUMAN HIBCH_HUMAN]] Defects in HIBCH are the cause of HIBCH deficiency (HIBCHD) [MIM:[http://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>
[[http://www.uniprot.org/uniprot/HIBCH_HUMAN HIBCH_HUMAN]] Defects in HIBCH are the cause of HIBCH deficiency (HIBCHD) [MIM:[http://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>
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[[Category: 3-hydroxyisobutyryl-CoA hydrolase]]
[[Category: 3-hydroxyisobutyryl-CoA hydrolase]]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Arrowsmith, C H.]]
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[[Category: Arrowsmith, C H]]
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[[Category: Delft, F von.]]
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[[Category: Delft, F von]]
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[[Category: Edwards, A M.]]
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[[Category: Edwards, A M]]
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[[Category: Guo, K.]]
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[[Category: Guo, K]]
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[[Category: King, O N.F.]]
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[[Category: King, O N.F]]
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[[Category: Oppermann, U.]]
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[[Category: Oppermann, U]]
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[[Category: Phillips, C.]]
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[[Category: Phillips, C]]
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[[Category: Pike, A C.W.]]
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[[Category: Pike, A C.W]]
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[[Category: Pilka, E S.]]
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[[Category: Pilka, E S]]
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[[Category: SGC, Structural Genomics Consortium.]]
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[[Category: Structural genomic]]
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[[Category: Weigelt, J.]]
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[[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]]
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[[Category: Structural genomics consortium]]
 
[[Category: Transit peptide]]
[[Category: Transit peptide]]

Revision as of 09:49, 20 January 2015

Crystal structure of human beta-hydroxyisobutyryl-CoA hydrolase in complex with quercetin

3bpt, resolution 1.50Å

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