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3dlx

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{{STRUCTURE_3dlx| PDB=3dlx | SCENE= }}
 
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===Crystal structure of human 3-oxoacid CoA transferase 1===
 
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==Disease==
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==Crystal structure of human 3-oxoacid CoA transferase 1==
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[[http://www.uniprot.org/uniprot/SCOT_HUMAN SCOT_HUMAN]] Defects in OXCT1 are a cause of succinyl-CoA-3-ketoacid-CoA transferase deficiency (SCOTD) [MIM:[http://omim.org/entry/245050 245050]]. A disorder of ketone body metabolism, characterized by episodic ketoacidosis. Patients are usually asymptomatic between episodes.<ref>PMID:10964512</ref><ref>PMID:9671268</ref><ref>PMID:21296660</ref>
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<StructureSection load='3dlx' size='340' side='right'caption='[[3dlx]], [[Resolution|resolution]] 2.20&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[3dlx]] is a 4 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=3DLX OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3DLX FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/SCOT_HUMAN SCOT_HUMAN]] Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate.
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.2&#8491;</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene></td></tr>
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==About this Structure==
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=3dlx FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3dlx OCA], [https://pdbe.org/3dlx PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3dlx RCSB], [https://www.ebi.ac.uk/pdbsum/3dlx PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3dlx ProSAT]</span></td></tr>
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[[3dlx]] is a 4 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=3DLX OCA].
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</table>
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== Disease ==
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==Reference==
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[https://www.uniprot.org/uniprot/SCOT1_HUMAN SCOT1_HUMAN] Succinyl-CoA:3-ketoacid CoA transferase deficiency. The disease is caused by mutations affecting the gene represented in this entry.
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<references group="xtra"/><references/>
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== Function ==
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[[Category: 3-oxoacid CoA-transferase]]
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[https://www.uniprot.org/uniprot/SCOT1_HUMAN SCOT1_HUMAN] Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate.
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/dl/3dlx_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</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=3dlx ConSurf].
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<div style="clear:both"></div>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Arrowsmith, C H.]]
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[[Category: Large Structures]]
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[[Category: Bountra, C.]]
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[[Category: Arrowsmith CH]]
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[[Category: Delft, F von.]]
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[[Category: Bountra C]]
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[[Category: Edwards, A M.]]
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[[Category: Edwards AM]]
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[[Category: Kavanagh, K L.]]
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[[Category: Kavanagh KL]]
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[[Category: Maclean, E M.]]
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[[Category: Maclean EM]]
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[[Category: Murray, J W.]]
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[[Category: Murray JW]]
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[[Category: Oppermann, U.]]
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[[Category: Oppermann U]]
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[[Category: Picaud, S.]]
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[[Category: Picaud S]]
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[[Category: Roos, A K.]]
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[[Category: Roos AK]]
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[[Category: SGC, Structural Genomics Consortium.]]
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[[Category: Shafqat N]]
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[[Category: Shafqat, N.]]
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[[Category: Wikstrom M]]
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[[Category: Wikstrom, M.]]
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[[Category: Yue WW]]
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[[Category: Yue, W W.]]
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[[Category: Von Delft F]]
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[[Category: Disease mutation]]
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[[Category: Mitochondrion]]
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[[Category: Oxct1]]
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[[Category: Scot]]
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[[Category: Sgc]]
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[[Category: Structural genomic]]
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[[Category: Structural genomics consortium]]
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[[Category: Succinyl-coa:3-ketoacid-coenzyme a transferase 1]]
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[[Category: Transferase]]
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[[Category: Transit peptide]]
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Current revision

Crystal structure of human 3-oxoacid CoA transferase 1

PDB ID 3dlx

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