2dnc: Difference between revisions
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{{STRUCTURE_2dnc| PDB=2dnc | SCENE= }} | {{STRUCTURE_2dnc| PDB=2dnc | SCENE= }} | ||
===Solution Structure of RSGI RUH-054, a lipoyl domain from human 2-oxoacid dehydrogenase=== | |||
=== | ==Disease== | ||
[[http://www.uniprot.org/uniprot/ODPX_HUMAN ODPX_HUMAN]] Defects in PDHX are the cause of pyruvate dehydrogenase E3-binding protein deficiency (PDHXD) [MIM:[http://omim.org/entry/245349 245349]].<ref>PMID:9399911</ref> | |||
==Function== | |||
[[http://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. | |||
==About this Structure== | ==About this Structure== | ||
| Line 11: | Line 13: | ||
==See Also== | ==See Also== | ||
*[[Pyruvate dehydrogenase|Pyruvate dehydrogenase]] | *[[Pyruvate dehydrogenase|Pyruvate dehydrogenase]] | ||
==Reference== | |||
<references group="xtra"/><references/> | |||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Hayashi, F.]] | [[Category: Hayashi, F.]] | ||
Revision as of 20:03, 24 March 2013
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
DiseaseDisease
[ODPX_HUMAN] Defects in PDHX are the cause of pyruvate dehydrogenase E3-binding protein deficiency (PDHXD) [MIM:245349].[1]
FunctionFunction
[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.
About this StructureAbout this Structure
2dnc is a 1 chain structure with sequence from Homo sapiens. Full experimental information is available from OCA.
See AlsoSee Also
ReferenceReference
- ↑ 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
Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)
OCACategories:
- Homo sapiens
- Hayashi, F.
- Hirota, H.
- Kurosaki, C.
- Momen, A Z.M Ruhul.
- RSGI, RIKEN Structural Genomics/Proteomics Initiative.
- Yokoyama, S.
- Yoshida, M.
- 2-oxoacid dehydrogenase
- Lipoic acid
- Lipoyl domain
- National project on protein structural and functional analyse
- Nppsfa
- Riken structural genomics/proteomics initiative
- Rsgi
- Structural genomic
- Transferase