3b8k

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[[Image:3b8k.jpg|left|200px]]
 
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==Structure of the Truncated Human Dihydrolipoyl Acetyltransferase (E2)==
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The line below this paragraph, containing "STRUCTURE_3b8k", creates the "Structure Box" on the page.
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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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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'>[[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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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">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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{{STRUCTURE_3b8k| PDB=3b8k | SCENE= }}
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</table>
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== Disease ==
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'''Structure of the Truncated Human Dihydrolipoyl Acetyltransferase (E2)'''
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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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== Function ==
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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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==Overview==
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== Evolutionary Conservation ==
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Dihydrolipoyl acetyltransferase (E2) is the central component of pyruvate dehydrogenase complex (PDC), which converts pyruvate to acetyl-CoA. Structural comparison by cryo-electron microscopy (cryo-EM) of the human full-length and truncated E2 (tE2) cores revealed flexible linkers emanating from the edges of trimers of the internal catalytic domains. Using the secondary structure constraints revealed in our 8 A cryo-EM reconstruction and the prokaryotic tE2 atomic structure as a template, we derived a pseudo atomic model of human tE2. The active sites are conserved between prokaryotic tE2 and human tE2. However, marked structural differences are apparent in the hairpin domain and in the N-terminal helix connected to the flexible linker. These permutations away from the catalytic center likely impart structures needed to integrate a second component into the inner core and provide a sturdy base for the linker that holds the pyruvate dehydrogenase for access by the E2-bound regulatory kinase/phosphatase components in humans.
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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==About this Structure==
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<jmolCheckbox>
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3B8K 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=3B8K OCA].
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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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==Reference==
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<text>to colour the structure by Evolutionary Conservation</text>
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Structures of the human pyruvate dehydrogenase complex cores: a highly conserved catalytic center with flexible N-terminal domains., Yu X, Hiromasa Y, Tsen H, Stoops JK, Roche TE, Zhou ZH, Structure. 2008 Jan;16(1):104-14. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/18184588 18184588]
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</jmolCheckbox>
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[[Category: Dihydrolipoyllysine-residue acetyltransferase]]
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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: Single protein]]
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[[Category: Large Structures]]
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[[Category: Hiromasa, Y.]]
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[[Category: Hiromasa Y]]
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[[Category: Roche, T E.]]
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[[Category: Roche TE]]
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[[Category: Stoops, J K.]]
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[[Category: Stoops JK]]
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[[Category: Tsen, H.]]
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[[Category: Tsen H]]
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[[Category: Yu, X.]]
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[[Category: Yu X]]
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[[Category: Zhou, Z H.]]
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[[Category: Zhou ZH]]
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[[Category: Central beta-sheet surrounded by five alpha-helice]]
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[[Category: Transferase]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun May 4 20:31:13 2008''
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Current revision

Structure of the Truncated Human Dihydrolipoyl Acetyltransferase (E2)

3b8k, resolution 8.80Å

Proteopedia Page Contributors and Editors (what is this?)

OCA

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