1zmc: Difference between revisions
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{{STRUCTURE_1zmc| PDB=1zmc | SCENE= }} | {{STRUCTURE_1zmc| PDB=1zmc | SCENE= }} | ||
===Crystal Structure of Human dihydrolipoamide dehydrogenase complexed to NAD+=== | |||
{{ABSTRACT_PUBMED_15946682}} | |||
=== | ==Disease== | ||
[[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. | |||
==Function== | |||
[[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== | ||
<ref group="xtra">PMID:015946682</ref><references group="xtra"/> | <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 11:39, 24 March 2013
Crystal Structure of Human dihydrolipoamide dehydrogenase complexed to NAD+
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
1zmc 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
