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lipoamide dehydrogenase (glycine cleavage system L protein, DLD, GCSL, LAD, LADH, PHE3)
Function:
- component of the glycine cleavage system
- E3 component of keto-acid dehydrogenases
Protein N(6)-(dihydrolipoyl)lysine + NAD+
protein N(6)-(lipoyl)lysine + NADH
Cofactor: binds 1 FAD per subunit (putative)
Structure:
- homodimer
- belongs to the class-1 pyridine nucleotide-disulfide oxidoreductase family
Compartment: mitochondria matrix
Pathology:
- defects in DLD are a cause of congenital infantile lactic acidosis
- defects in DLD are a cause of maple syrup urine disease
Related
DLD gene mutation
General
dehydrogenase
diaphorase
Properties
SIZE: entity length = 509 aa
MW = 54 kD
COMPARTMENT: mitochondria
MOTIF: cofactor-binding site [71-80]
COFACTOR-BOUND: flavin adenine dinucleotide
MOTIF: cysteine residue {C80}
MODIFICATION: cysteine residue {C85}
cysteine residue {C85}
MODIFICATION: cysteine residue {C80}
histidine residue {H487}
Database Correlations
OMIM 246900
UniProt P09622
ENZYME correlations
References
- OMIM :accession 246900 & 248600
- UniProt :accession P09622
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=DLD
Component-of
alpha-ketoglutarate dehydrogenase