3b8k

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{{STRUCTURE_3b8k| PDB=3b8k | SCENE= }}
 
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===Structure of the Truncated Human Dihydrolipoyl Acetyltransferase (E2)===
 
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{{ABSTRACT_PUBMED_18184588}}
 
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==Disease==
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==Structure of the Truncated Human Dihydrolipoyl Acetyltransferase (E2)==
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[[http://www.uniprot.org/uniprot/ODP2_HUMAN ODP2_HUMAN]] Note=Primary biliary cirrhosis is a chronic, progressive cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients' serum. It manifests with inflammatory obliteration of intra-hepatic bile duct, leading to liver cell damage and cirrhosis. Patients with primary biliary cirrhosis show autoantibodies against the E2 component of pyruvate dehydrogenase complex. Defects in DLAT are the cause of pyruvate dehydrogenase E2 deficiency (PDHE2 deficiency) [MIM:[http://omim.org/entry/245348 245348]]; also known as lactic acidemia due to defect of E2 lipoyl transacetylase of the pyruvate dehydrogenase complex. Pyruvate dehydrogenase (PDH) deficiency is a major cause of primary lactic acidosis and neurological dysfunction in infancy and early childhood. In this form of PDH deficiency episodic dystonia is the major neurological manifestation, with other more common features of pyruvate dehydrogenase deficiency, such as hypotonia and ataxia, being less prominent.
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<SX load='3b8k' size='340' side='right' viewer='molstar' caption='[[3b8k]], [[Resolution|resolution]] 8.80&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[3b8k]] is a 1 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=3B8K OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3B8K FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/ODP2_HUMAN ODP2_HUMAN]] The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 8.8&#8491;</td></tr>
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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=3b8k FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3b8k OCA], [https://pdbe.org/3b8k PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3b8k RCSB], [https://www.ebi.ac.uk/pdbsum/3b8k PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3b8k ProSAT]</span></td></tr>
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==About this Structure==
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</table>
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[[3b8k]] is a 1 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=3B8K OCA].
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== Disease ==
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[https://www.uniprot.org/uniprot/ODP2_HUMAN ODP2_HUMAN] Note=Primary biliary cirrhosis is a chronic, progressive cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients' serum. It manifests with inflammatory obliteration of intra-hepatic bile duct, leading to liver cell damage and cirrhosis. Patients with primary biliary cirrhosis show autoantibodies against the E2 component of pyruvate dehydrogenase complex. Defects in DLAT are the cause of pyruvate dehydrogenase E2 deficiency (PDHE2 deficiency) [MIM:[https://omim.org/entry/245348 245348]; also known as lactic acidemia due to defect of E2 lipoyl transacetylase of the pyruvate dehydrogenase complex. Pyruvate dehydrogenase (PDH) deficiency is a major cause of primary lactic acidosis and neurological dysfunction in infancy and early childhood. In this form of PDH deficiency episodic dystonia is the major neurological manifestation, with other more common features of pyruvate dehydrogenase deficiency, such as hypotonia and ataxia, being less prominent.
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==See Also==
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== Function ==
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*[[Pyruvate dehydrogenase|Pyruvate dehydrogenase]]
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[https://www.uniprot.org/uniprot/ODP2_HUMAN ODP2_HUMAN] The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.
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== Evolutionary Conservation ==
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==Reference==
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[[Image:Consurf_key_small.gif|200px|right]]
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<ref group="xtra">PMID:018184588</ref><references group="xtra"/><references/>
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Check<jmol>
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[[Category: Dihydrolipoyllysine-residue acetyltransferase]]
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/b8/3b8k_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=3b8k ConSurf].
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<div style="clear:both"></div>
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__TOC__
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</SX>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Hiromasa, Y.]]
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[[Category: Large Structures]]
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[[Category: Roche, T E.]]
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[[Category: Hiromasa Y]]
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[[Category: Stoops, J K.]]
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[[Category: Roche TE]]
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[[Category: Tsen, H.]]
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[[Category: Stoops JK]]
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[[Category: Yu, X.]]
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[[Category: Tsen H]]
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[[Category: Zhou, Z H.]]
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[[Category: Yu X]]
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[[Category: Central beta-sheet surrounded by five alpha-helice]]
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[[Category: Zhou ZH]]
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[[Category: Transferase]]
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Current revision

Structure of the Truncated Human Dihydrolipoyl Acetyltransferase (E2)

3b8k, resolution 8.80Å

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OCA

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