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[[Image:2dnc.jpg|left|200px]]<br /><applet load="2dnc" size="350" color="white" frame="true" align="right" spinBox="true"
caption="2dnc" />
'''Solution Structure of RSGI RUH-054, a lipoyl domain from human 2-oxoacid dehydrogenase'''<br />


==Disease==
==Solution Structure of RSGI RUH-054, a lipoyl domain from human 2-oxoacid dehydrogenase==
Known disease associated with this structure: Lacticacidemia due to PDX1 deficiency OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=608769 608769]]
<StructureSection load='2dnc' size='340' side='right'caption='[[2dnc]]' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[2dnc]] 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=2DNC OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2DNC FirstGlance]. <br>
</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=2dnc FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2dnc OCA], [https://pdbe.org/2dnc PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2dnc RCSB], [https://www.ebi.ac.uk/pdbsum/2dnc PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2dnc ProSAT], [https://www.topsan.org/Proteins/RSGI/2dnc TOPSAN]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/ODPX_HUMAN ODPX_HUMAN] Defects in PDHX are the cause of pyruvate dehydrogenase E3-binding protein deficiency (PDHXD) [MIM:[https://omim.org/entry/245349 245349].<ref>PMID:9399911</ref>
== Function ==
[https://www.uniprot.org/uniprot/ODPX_HUMAN ODPX_HUMAN] Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
  <jmolCheckbox>
    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/dn/2dnc_consurf.spt"</scriptWhenChecked>
    <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=2dnc ConSurf].
<div style="clear:both"></div>


==About this Structure==
==See Also==
2DNC is a [http://en.wikipedia.org/wiki/Single_protein 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=2DNC OCA].
*[[Pyruvate dehydrogenase 3D structures|Pyruvate dehydrogenase 3D structures]]
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Single protein]]
[[Category: Large Structures]]
[[Category: Hayashi, F.]]
[[Category: Hayashi F]]
[[Category: Hirota, H.]]
[[Category: Hirota H]]
[[Category: Kurosaki, C.]]
[[Category: Kurosaki C]]
[[Category: Momen, A Z.M Ruhul.]]
[[Category: Ruhul Momen AZM]]
[[Category: RSGI, RIKEN Structural Genomics/Proteomics Initiative.]]
[[Category: Yokoyama S]]
[[Category: Yokoyama, S.]]
[[Category: Yoshida M]]
[[Category: Yoshida, M.]]
[[Category: 2-oxoacid dehydrogenase]]
[[Category: lipoic acid]]
[[Category: lipoyl domain]]
[[Category: national project on protein structural and functional analyses]]
[[Category: nppsfa]]
[[Category: riken structural genomics/proteomics initiative]]
[[Category: rsgi]]
[[Category: structural genomics]]
 
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 17:00:37 2008''

Latest revision as of 21:44, 29 May 2024

Solution Structure of RSGI RUH-054, a lipoyl domain from human 2-oxoacid dehydrogenaseSolution Structure of RSGI RUH-054, a lipoyl domain from human 2-oxoacid dehydrogenase

Structural highlights

2dnc is a 1 chain structure with sequence from Homo sapiens. Full experimental information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:Solution NMR
Resources:FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT, TOPSAN

Disease

ODPX_HUMAN Defects in PDHX are the cause of pyruvate dehydrogenase E3-binding protein deficiency (PDHXD) [MIM:245349.[1]

Function

ODPX_HUMAN Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.

Evolutionary Conservation

Check, as determined by ConSurfDB. You may read the explanation of the method and the full data available from ConSurf.

See Also

References

  1. ↑ Aral B, Benelli C, Ait-Ghezala G, Amessou M, Fouque F, Maunoury C, Creau N, Kamoun P, Marsac C. Mutations in PDX1, the human lipoyl-containing component X of the pyruvate dehydrogenase-complex gene on chromosome 11p1, in congenital lactic acidosis. Am J Hum Genet. 1997 Dec;61(6):1318-26. PMID:9399911 doi:S0002-9297(07)60233-X
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