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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; 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; HAD1; HADH; HADH1; HADHSC; HCDH; HHF4; MSCHAD; SCHAD; wu:fb66a11; zgc:86777
UniProt ID: (Human) Q16836, (Mouse) Q61425
Entrez Gene ID: (Human) 3033, (Mouse) 15107, (Zebrafish) 445121
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