Lipoyltransferase 1 (LIPT1)

In mammals, lipoyltransferase (EC 2.3.1.181) catalyzes the second of 2 steps in the covalent attachment of lipoic acid to lipoate-dependent proteins, including the glycine cleavage system H protein (GCSH) and the dihydrolipoamide acyltransferase (E2) subunits of the pyruvate dehydrogenase complex (DLAT), the 2-oxoglutarate dehydrogenase complex (DLST), and the branched-chain 2-oxo acid dehydrogenase complex (DBT) .The deduced 373-amino acid precursor protein shares 88% and 31% sequence identity with bovine lipoyltransferase II and E. coli lipoate-protein ligase A, respectively. LIPT1 enzyme contains 347 amino acids with a calculated molecular mass of 39.539 kD. transcript in all tissues examined, with highest expression in skeletal muscle and heart, followed by liver, brain, placenta, and lung.