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2coo

From Proteopedia

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(New page: 200px<br /> <applet load="2coo" size="450" color="white" frame="true" align="right" spinBox="true" caption="2coo" /> '''Solution structure of the e3_binding domain...)
Current revision (11:27, 22 May 2024) (edit) (undo)
 
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[[Image:2coo.gif|left|200px]]<br />
 
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<applet load="2coo" size="450" color="white" frame="true" align="right" spinBox="true"
 
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caption="2coo" />
 
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'''Solution structure of the e3_binding domain of dihydrolipoamide branched chaintransacylase'''<br />
 
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==Disease==
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==Solution structure of the e3_binding domain of dihydrolipoamide branched chaintransacylase==
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Known diseases associated with this structure: Maple syrup urine disease, type II OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=248610 248610]]
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<StructureSection load='2coo' size='340' side='right'caption='[[2coo]]' scene=''>
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== Structural highlights ==
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==About this Structure==
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<table><tr><td colspan='2'>[[2coo]] 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=2COO OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2COO FirstGlance]. <br>
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2COO is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Active as [http://en.wikipedia.org/wiki/Dihydrolipoyllysine-residue_(2-methylpropanoyl)transferase Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.3.1.168 2.3.1.168] Full crystallographic information is available from [http://ispc.weizmann.ac.il/oca-bin/ocashort?id=2COO OCA].
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr>
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[[Category: Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]]
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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=2coo FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2coo OCA], [https://pdbe.org/2coo PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2coo RCSB], [https://www.ebi.ac.uk/pdbsum/2coo PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2coo ProSAT], [https://www.topsan.org/Proteins/RSGI/2coo TOPSAN]</span></td></tr>
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</table>
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== Disease ==
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[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>
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== Function ==
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[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).
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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/co/2coo_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=2coo ConSurf].
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<div style="clear:both"></div>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Hayashi, F.]]
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[[Category: Hayashi F]]
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[[Category: RSGI, RIKEN.Structural.Genomics/Proteomics.Initiative.]]
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[[Category: Yokoyama S]]
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[[Category: Yokoyama, S.]]
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[[Category: Zhang HP]]
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[[Category: Zhang, H.P.]]
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[[Category: dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]]
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[[Category: e3_binding domain]]
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[[Category: lipoamide acyltransferase component of branched-chain alpha-keto acid]]
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[[Category: national project on protein structural and functional analyses]]
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[[Category: nppsfa]]
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[[Category: riken structural genomics/proteomics initiative]]
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[[Category: rsgi]]
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[[Category: structural genomics]]
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''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Mon Nov 12 21:18:34 2007''
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Current revision

Solution structure of the e3_binding domain of dihydrolipoamide branched chaintransacylase

PDB ID 2coo

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