Hydroxyacyl-Coenzyme A dehydrogenase

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Hydroxyacyl-CoA dehydrogenase
PDB 3had EBI.jpg
PDB rendering based on 3had.
Available structures
PDB Ortholog search: PDBe, RCSB
External IDs OMIM601609 MGI96009 HomoloGene55888 GeneCards: HADH Gene
EC number
Species Human Mouse
Entrez 3033 15107
Ensembl ENSG00000138796 ENSMUSG00000027984
UniProt Q16836 Q61425
RefSeq (mRNA) NM_001184705 NM_008212
RefSeq (protein) NP_001171634 NP_032238
Location (UCSC) Chr 4:
108.91 – 108.96 Mb
Chr 3:
131.23 – 131.27 Mb
PubMed search [1] [2]

Hydroxyacyl-Coenzyme A dehydrogenase also known as HADH is an enzyme which in humans is encoded by the HADH gene.[1][2]


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.[3]

Clinical significance[edit]

Mutations in this gene cause one form of familial hyperinsulinemic hypoglycemia.[4] A deficiency is associated with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.

See also[edit]


  1. ^ Craig I, Tolley E, Bobrow M (1976). "A preliminary analysis of the segregation of human hydroxyacyl coenzyme A dehydrogenase in human-mouse somatic cell hybrids". Cytogenet. Cell Genet. 16 (1–5): 114–7. doi:10.1159/000130568. PMID 975867. 
  2. ^ Yang SY, He XY, Schulz H (October 2005). "3-Hydroxyacyl-CoA dehydrogenase and short chain 3-hydroxyacyl-CoA dehydrogenase in human health and disease". FEBS J. 272 (19): 4874–83. doi:10.1111/j.1742-4658.2005.04911.x. PMID 16176262. 
  3. ^ "Entrez Gene: HADH". 
  4. ^ Molven A, Matre GE, Duran M, Wanders RJ, Rishaug U, Njølstad PR, Jellum E, Søvik O (January 2004). "Familial hyperinsulinemic hypoglycemia caused by a defect in the SCHAD enzyme of mitochondrial fatty acid oxidation". Diabetes 53 (1): 221–7. doi:10.2337/diabetes.53.1.221. PMID 14693719. 

External links[edit]

This article incorporates text from the United States National Library of Medicine, which is in the public domain.