The coenzyme A thioester derivative of propionic acid; an intermediate in the degradation of l-valine, l-isoleucine, l-threonine, l-methionine, and odd-chain fatty acid s; a precursor for the synthesis of odd-chain fatty acid s; it accumulates in individuals with a deficiency of propionyl-CoA carboxylase.
- propionyl-CoA carboxylase an enzyme that catalyzes the reaction of propionyl-CoA with CO2 and ATP to produce ADP, orthophosphate, and d-methylmalonyl-CoA; a biotin-dependent enzyme; an inherited deficiency of this enzyme will lead to propionic acidemia and developmental retardation.
Medical dictionary. 2011.