This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase gene family. The encoded protein 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 of this gene on chromosome 15.
HADH; hydroxyacyl-CoA dehydrogenase; HADHSC, hydroxyacyl Coenzyme A dehydrogenase , L 3 hydroxyacyl Coenzyme A dehydrogenase, short chain; hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; HADH1; SCHAD; short-chain 3-hydroxyacyl-CoA dehydrogenase; L-; Hadhsc; hydroxyacyl-Coenzyme A dehydrogenase; zgc:86777; wu:fb66a11; L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain