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

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.

References

↑ 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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OCA

[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

[1]

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

Structural highlights

Disease

Function

Evolutionary Conservation

See Also

References

Contents

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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

Structural highlights

Disease

Function

Evolutionary Conservation

See Also

References

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