2dne
From Proteopedia
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==Solution Structure of RSGI RUH-058, a lipoyl domain of human 2-oxoacid dehydrogenase== | ==Solution Structure of RSGI RUH-058, a lipoyl domain of human 2-oxoacid dehydrogenase== | ||
| - | <StructureSection load='2dne' size='340' side='right'caption='[[2dne | + | <StructureSection load='2dne' size='340' side='right'caption='[[2dne]]' scene=''> |
== Structural highlights == | == Structural highlights == | ||
| - | <table><tr><td colspan='2'>[[2dne]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/ | + | <table><tr><td colspan='2'>[[2dne]] 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=2DNE OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2DNE FirstGlance]. <br> |
| - | </td></tr><tr id=' | + | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr> |
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2dne FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2dne OCA], [https://pdbe.org/2dne PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2dne RCSB], [https://www.ebi.ac.uk/pdbsum/2dne PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2dne ProSAT], [https://www.topsan.org/Proteins/RSGI/2dne TOPSAN]</span></td></tr> | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2dne FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2dne OCA], [https://pdbe.org/2dne PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2dne RCSB], [https://www.ebi.ac.uk/pdbsum/2dne PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2dne ProSAT], [https://www.topsan.org/Proteins/RSGI/2dne TOPSAN]</span></td></tr> | ||
</table> | </table> | ||
== Disease == | == Disease == | ||
| - | + | [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. | |
== Function == | == Function == | ||
| - | + | [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. | |
== Evolutionary Conservation == | == Evolutionary Conservation == | ||
[[Image:Consurf_key_small.gif|200px|right]] | [[Image:Consurf_key_small.gif|200px|right]] | ||
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__TOC__ | __TOC__ | ||
</StructureSection> | </StructureSection> | ||
| - | [[Category: | + | [[Category: Homo sapiens]] |
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[[Category: Large Structures]] | [[Category: Large Structures]] | ||
| - | [[Category: Hayashi | + | [[Category: Hayashi F]] |
| - | [[Category: Hirota | + | [[Category: Hirota H]] |
| - | [[Category: Momen | + | [[Category: Ruhul Momen AZM]] |
| - | + | [[Category: Yokoyama S]] | |
| - | [[Category: Yokoyama | + | |
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Current revision
Solution Structure of RSGI RUH-058, a lipoyl domain of human 2-oxoacid dehydrogenase
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