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2dnc

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[[Image:2dnc.png|left|200px]]
 
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{{STRUCTURE_2dnc| PDB=2dnc | SCENE= }}
{{STRUCTURE_2dnc| PDB=2dnc | SCENE= }}
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===Solution Structure of RSGI RUH-054, a lipoyl domain from human 2-oxoacid dehydrogenase===
===Solution Structure of RSGI RUH-054, a lipoyl domain from human 2-oxoacid dehydrogenase===
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==Disease==
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[[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>
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==Function==
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[[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==
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==See Also==
==See Also==
*[[Pyruvate dehydrogenase|Pyruvate dehydrogenase]]
*[[Pyruvate dehydrogenase|Pyruvate dehydrogenase]]
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==Reference==
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<references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Hayashi, F.]]
[[Category: Hayashi, F.]]

Revision as of 17:03, 24 March 2013

Template:STRUCTURE 2dnc

Contents

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

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.

About this Structure

2dnc is a 1 chain structure with sequence from Homo sapiens. Full experimental information is available from OCA.

See Also

Reference

  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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OCA

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