1dtw

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{{Seed}}
 
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[[Image:1dtw.png|left|200px]]
 
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==HUMAN BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE==
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The line below this paragraph, containing "STRUCTURE_1dtw", creates the "Structure Box" on the page.
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<StructureSection load='1dtw' size='340' side='right'caption='[[1dtw]], [[Resolution|resolution]] 2.70&Aring;' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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<table><tr><td colspan='2'>[[1dtw]] is a 2 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=1DTW OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1DTW FirstGlance]. <br>
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or leave the SCENE parameter empty for the default display.
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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]] 2.7&#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=K:POTASSIUM+ION'>K</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=TPP:THIAMINE+DIPHOSPHATE'>TPP</scene></td></tr>
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{{STRUCTURE_1dtw| PDB=1dtw | SCENE= }}
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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=1dtw FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1dtw OCA], [https://pdbe.org/1dtw PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1dtw RCSB], [https://www.ebi.ac.uk/pdbsum/1dtw PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1dtw ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/ODBA_HUMAN ODBA_HUMAN] Defects in BCKDHA are a cause of maple syrup urine disease type IA (MSUD1A) [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:2060625</ref> <ref>PMID:8037208</ref> <ref>PMID:2703538</ref> <ref>PMID:2241958</ref> <ref>PMID:1867199</ref> <ref>PMID:1885764</ref> <ref>PMID:8161368</ref> <ref>PMID:7883996</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/ODBA_HUMAN ODBA_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).
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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/dt/1dtw_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=1dtw ConSurf].
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<div style="clear:both"></div>
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===HUMAN BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE===
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==See Also==
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*[[2-oxoisovalerate dehydrogenase 3D structures|2-oxoisovalerate dehydrogenase 3D structures]]
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== References ==
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<references/>
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The line below this paragraph, {{ABSTRACT_PUBMED_10745006}}, adds the Publication Abstract to the page
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__TOC__
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(as it appears on PubMed at http://www.pubmed.gov), where 10745006 is the PubMed ID number.
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</StructureSection>
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{{ABSTRACT_PUBMED_10745006}}
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==About this Structure==
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1DTW is a [[Protein complex]] structure of sequences 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=1DTW OCA].
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==Reference==
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Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease., AEvarsson A, Chuang JL, Wynn RM, Turley S, Chuang DT, Hol WG, Structure. 2000 Mar 15;8(3):277-91. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/10745006 10745006]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Protein complex]]
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[[Category: Large Structures]]
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[[Category: AEvarsson, A.]]
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[[Category: AEvarsson A]]
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[[Category: Chuang, D T.]]
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[[Category: Chuang DT]]
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[[Category: Chuang, J L.]]
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[[Category: Chuang JL]]
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[[Category: Hol, W G.J.]]
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[[Category: Hol WGJ]]
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[[Category: Turley, S.]]
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[[Category: Turley S]]
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[[Category: Wynn, R M.]]
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[[Category: Wynn RM]]
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[[Category: Branched-chain alpha-keto acid dehydrogenase]]
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[[Category: Thdp-binding fold]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Jun 30 23:36:36 2008''
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Current revision

HUMAN BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE

PDB ID 1dtw

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