DLD

Lipoamide dehydrogenase or dihydrolipoamide dehydrogenase (DLD) is a component of the glycine cleavage system as well as of the alpha ketoacid dehydrogenase complexes. It is a homodimer. It belongs to the class I pyridine nucleotide disulfide oxidoreductase family. Defects in DLD are a cause of congenital infantile lactic acidosis. Moreover, defects in DLD are the cause of DLD deficiency; which results in extensive metabolic disturbances, including lactic acidemia, Krebs cycle dysfunction, and impaired branched amino acid degradation. DLD deficiency also causes neurological degeneration due to the sensitivity of the central nervous system to defects in oxidative metabolism.