1dtw

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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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__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 2 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=1DTW OCA].
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==Reference==
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<ref group="xtra">PMID:010745006</ref><ref group="xtra">PMID:011509994</ref><ref group="xtra">PMID:012651851</ref><references group="xtra"/>
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: AEvarsson, A.]]
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[[Category: Large Structures]]
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[[Category: Chuang, D T.]]
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[[Category: AEvarsson A]]
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[[Category: Chuang, J L.]]
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[[Category: Chuang DT]]
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[[Category: Hol, W G.J.]]
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[[Category: Chuang JL]]
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[[Category: Turley, S.]]
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[[Category: Hol WGJ]]
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[[Category: Wynn, R M.]]
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[[Category: Turley S]]
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[[Category: Branched-chain alpha-keto acid dehydrogenase]]
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[[Category: Wynn RM]]
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[[Category: Oxidoreductase]]
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[[Category: Thdp-binding fold]]
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Current revision

HUMAN BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE

PDB ID 1dtw

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