1zmd

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[[Image:1zmd.png|left|200px]]
 
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{{STRUCTURE_1zmd| PDB=1zmd | SCENE= }}
{{STRUCTURE_1zmd| PDB=1zmd | SCENE= }}
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===Crystal Structure of Human dihydrolipoamide dehydrogenase complexed to NADH===
===Crystal Structure of Human dihydrolipoamide dehydrogenase complexed to NADH===
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{{ABSTRACT_PUBMED_15946682}}
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{{ABSTRACT_PUBMED_15946682}}
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==Disease==
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[[http://www.uniprot.org/uniprot/DLDH_HUMAN DLDH_HUMAN]] Note=Defects in DLD are involved in the development of congenital infantile lactic acidosis. Defects in DLD are a cause of maple syrup urine disease (MSUD) [MIM:[http://omim.org/entry/248600 248600]]. MSUD is characterized by mental and physical retardation, feeding problems and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine, resulting from a block in oxidative decarboxylation.
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==Function==
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[[http://www.uniprot.org/uniprot/DLDH_HUMAN DLDH_HUMAN]] Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
==About this Structure==
==About this Structure==
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==Reference==
==Reference==
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<ref group="xtra">PMID:015946682</ref><references group="xtra"/>
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<ref group="xtra">PMID:015946682</ref><references group="xtra"/><references/>
[[Category: Dihydrolipoyl dehydrogenase]]
[[Category: Dihydrolipoyl dehydrogenase]]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]

Revision as of 23:38, 24 March 2013

Template:STRUCTURE 1zmd

Contents

Crystal Structure of Human dihydrolipoamide dehydrogenase complexed to NADH

Template:ABSTRACT PUBMED 15946682

Disease

[DLDH_HUMAN] Note=Defects in DLD are involved in the development of congenital infantile lactic acidosis. Defects in DLD are a cause of maple syrup urine disease (MSUD) [MIM:248600]. MSUD is characterized by mental and physical retardation, feeding problems and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine, resulting from a block in oxidative decarboxylation.

Function

[DLDH_HUMAN] Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.

About this Structure

1zmd is a 8 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

See Also

Reference

  • Brautigam CA, Chuang JL, Tomchick DR, Machius M, Chuang DT. Crystal structure of human dihydrolipoamide dehydrogenase: NAD+/NADH binding and the structural basis of disease-causing mutations. J Mol Biol. 2005 Jul 15;350(3):543-52. PMID:15946682 doi:10.1016/j.jmb.2005.05.014

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OCA

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