Description
Product Characteristics:
Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
Subcellular location: Cytoplasm
Synonyms: HAD, HCDH, HHF4, HADH1, SCHAD, HADHSC, MSCHAD, Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial, Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase, Short-chain 3-hydroxyacyl-CoA dehydrogenase, HADH
Target Information: 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. [provided by RefSeq, May 2010]