1zmd

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Template:STRUCTURE 1zmd

Contents 1 Crystal Structure of Human dihydrolipoamide dehydrogenase complexed to NADH 2 Disease 3 Function 4 About this Structure 5 See Also 6 Reference

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

• Dihydrolipoamide dehydrogenase

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