HAD, HADHSC, HCDH, HHF4, M SCHAD, Medium and short chain L 3 hydroxyacyl coenzyme A dehydrogenase, SCHAD, Short chain 3 hydroxyacyl CoA dehydrogenase mitochondrial.
HADHSC functions in the mitochondrial matrix to catalyze the oxidation of straight-chain 3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. Its enzymatic activity is highest with medium-chain-length fatty acids. Mutations in this gene cause one form of familial hyperinsulinemic hypoglycemia. The human genome contains a related pseudogene.