1k8m: Difference between revisions
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==Solution Structure of the Lipoic Acid-Bearing Domain of the E2 component of Human, Mitochondrial Branched-Chain alpha-Ketoacid Dehydrogenase== | ==Solution Structure of the Lipoic Acid-Bearing Domain of the E2 component of Human, Mitochondrial Branched-Chain alpha-Ketoacid Dehydrogenase== | ||
<StructureSection load='1k8m' size='340' side='right'caption='[[1k8m | <StructureSection load='1k8m' size='340' side='right'caption='[[1k8m]]' scene=''> | ||
== Structural highlights == | == Structural highlights == | ||
<table><tr><td colspan='2'>[[1k8m]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/ | <table><tr><td colspan='2'>[[1k8m]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1K8M OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1K8M FirstGlance]. <br> | ||
</td></tr> | </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=1k8m FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1k8m OCA], [https://pdbe.org/1k8m PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1k8m RCSB], [https://www.ebi.ac.uk/pdbsum/1k8m PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1k8m ProSAT]</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=1k8m FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1k8m OCA], [https://pdbe.org/1k8m PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1k8m RCSB], [https://www.ebi.ac.uk/pdbsum/1k8m PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1k8m ProSAT]</span></td></tr> | |||
</table> | </table> | ||
== Disease == | == Disease == | ||
[https://www.uniprot.org/uniprot/ODB2_HUMAN ODB2_HUMAN] Defects in DBT are the cause of maple syrup urine disease type 2 (MSUD2) [MIM:[https://omim.org/entry/248600 248600]. MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine.<ref>PMID:1847055</ref> <ref>PMID:9621512</ref> | |||
== Function == | == Function == | ||
[https://www.uniprot.org/uniprot/ODB2_HUMAN ODB2_HUMAN] The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). | |||
== Evolutionary Conservation == | == Evolutionary Conservation == | ||
[[Image:Consurf_key_small.gif|200px|right]] | [[Image:Consurf_key_small.gif|200px|right]] | ||
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__TOC__ | __TOC__ | ||
</StructureSection> | </StructureSection> | ||
[[Category: | [[Category: Homo sapiens]] | ||
[[Category: Large Structures]] | [[Category: Large Structures]] | ||
[[Category: Chang | [[Category: Chang C-F]] | ||
[[Category: Chou | [[Category: Chou H-T]] | ||
[[Category: Chuang | [[Category: Chuang DT]] | ||
[[Category: Chuang | [[Category: Chuang JL]] | ||
[[Category: Huang | [[Category: Huang T-h]] | ||