3bpt
From Proteopedia
(Difference between revisions)
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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> | ||
| - | </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>< | + | </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> |
| - | <tr><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</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> |
| - | <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> | + | <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> |
| - | <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> | + | <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> |
| - | <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> | + | <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> |
| - | <table> | + | </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]] | ||
| - | [[Category: Arrowsmith, C H | + | [[Category: Arrowsmith, C H]] |
| - | [[Category: Delft, F von | + | [[Category: Delft, F von]] |
| - | [[Category: Edwards, A M | + | [[Category: Edwards, A M]] |
| - | [[Category: Guo, K | + | [[Category: Guo, K]] |
| - | [[Category: King, O N.F | + | [[Category: King, O N.F]] |
| - | [[Category: Oppermann, U | + | [[Category: Oppermann, U]] |
| - | [[Category: Phillips, C | + | [[Category: Phillips, C]] |
| - | [[Category: Pike, A C.W | + | [[Category: Pike, A C.W]] |
| - | [[Category: Pilka, E S | + | [[Category: Pilka, E S]] |
| - | [[Category: | + | [[Category: Structural genomic]] |
| - | [[Category: Weigelt, J | + | [[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]] | ||
Revision as of 09:49, 20 January 2015
Crystal structure of human beta-hydroxyisobutyryl-CoA hydrolase in complex with quercetin
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Categories: 3-hydroxyisobutyryl-CoA hydrolase | Homo sapiens | 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
