2dnc
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
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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