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

More general terms

• diaphorase
• dehydrogenase

Component of

• pyruvate dehydrogenase

Database

• UniProt: http://www.uniprot.org/uniprot/P09622.html
• OMIM: https://mirror.omim.org/entry/246900