HADH, Official Symbol HADHprovided by HGNC Official Full Name hydroxyacyl-CoA dehydrogenaseprovided by HGNC Primary source HGNC:HGNC:4799 See related Ensembl:ENSG00000138796 MIM:601609; AllianceGenome:HGNC:4799 Gene type protein coding RefSeq status REVIEWED Organism Homo sapiens Lineage Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo Also known as HAD; HCDH; HHF4; HADH1; SCHAD; HADHSC; MSCHAD Summary 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] Expression Ubiquitous expression in fat (RPKM 86.5), kidney (RPKM 61.3) and 24 other tissues See more Orthologs mouse all