Sequence of this protein is as follows: MAFVTRQFMR SVSSSSTASA SAKKIIVKHV TVIGGGLMGA GIAQVAAATG HTVVLVDQTE DILAKSKKGI EESLRKVAKK KFAENPKAGD EFVEKTLSTI ATSTDAASVV HSTDLVVEAI VENLKVKNEL FKRLDKFAAE HTIFASNTSS LQITSIANAT TRQDRFAGLH FFNPVPVMKL VEVIKTPMTS QKTFESLVDF SKALGKHPVS CKDTPGFIVN RLLVPYLMEA IRLYERGDAS KEDIDTAMKL GAGYPMGPFE LLDYVGLDTT KFIVDGWHEM DAENPLHQPS PSLNKLVAEN KFGKKTGEGF YKYK
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; hydroxylacyl-Coenzyme A dehydrogenase-dehydrogenase; L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain; M/SCHAD; medium and short chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; MGC8392; short chain 3-hydroxyacyl-CoA dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase; testis secretory sperm-binding protein Li 203a
Gene Aliases: AA409008; AU019341; AW742602; HAD; HADH; HADH1; HADHSC; HCDH; HHF4; MSCHAD; SCHAD