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[[Image:2coo.gif|left|200px]]


{{Structure
==Solution structure of the e3_binding domain of dihydrolipoamide branched chaintransacylase==
|PDB= 2coo |SIZE=350|CAPTION= <scene name='initialview01'>2coo</scene>
<StructureSection load='2coo' size='340' side='right'caption='[[2coo]]' scene=''>
|SITE=  
== Structural highlights ==
|LIGAND=  
<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>
|ACTIVITY= [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]  
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr>
|GENE= DBT, BCATE2 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
<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>
}}
</table>
 
== Disease ==
'''Solution structure of the e3_binding domain of dihydrolipoamide branched chaintransacylase'''
[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 ==
 
[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).
==Disease==
== Evolutionary Conservation ==
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]]
[[Image:Consurf_key_small.gif|200px|right]]
 
Check<jmol>
==About this Structure==
  <jmolCheckbox>
2COO is a [[Single protein]] structure of 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=2COO OCA].  
    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/co/2coo_consurf.spt"</scriptWhenChecked>
[[Category: Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]]
    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
    <text>to colour the structure by Evolutionary Conservation</text>
  </jmolCheckbox>
</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].
<div style="clear:both"></div>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Single protein]]
[[Category: Large Structures]]
[[Category: Hayashi, F.]]
[[Category: Hayashi F]]
[[Category: RSGI, RIKEN Structural Genomics/Proteomics Initiative.]]
[[Category: Yokoyama S]]
[[Category: Yokoyama, S.]]
[[Category: Zhang HP]]
[[Category: Zhang, H P.]]
[[Category: dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]]
[[Category: e3_binding domain]]
[[Category: lipoamide acyltransferase component of branched-chain alpha-keto acid]]
[[Category: national project on protein structural and functional analyse]]
[[Category: nppsfa]]
[[Category: riken structural genomics/proteomics initiative]]
[[Category: rsgi]]
[[Category: structural genomic]]
 
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 16:18:05 2008''
Retrieved from "https://proteopedia.org/2coo"