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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.
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Protein Aliases: HCDH; Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain; M/SCHAD; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; MGC8392; Short-chain 3-hydroxyacyl-CoA dehydrogenase; testis secretory sperm-binding protein Li 203a
Gene Aliases: HAD; HAD1; HADH; HADH1; HADHSC; HCDH; HHF4; MSCHAD; SCHAD
UniProt ID: (Human) Q16836
Entrez Gene ID: (Human) 3033