HADH (Hydroxyacyl-CoA Dehydrogenase, also known as HADHSC) is a Protein Coding gene. 2 alternatively spliced human isoforms have been reported. 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. HADH is a key enzyme in fatty acid oxidation. It is widely expressed in fat, kidney, and other tissues. The downregulation of HADH promotes gastric cancer progression via activation of the Akt signaling pathway. Recessive mutations in the hydroxyacyl-CoA dehydrogenase (HADH) gene encoding the enzyme 3-hydroxyacyl-CoA dehydrogenase are a rare cause of diazoxide-responsive hyperinsulinemic hypoglycemia (HH).