Gene Symbol: Hadh
Description: hydroxyacyl-Coenzyme A dehydrogenase
Alias: AA409008, AU019341, AW742602, HCDH, Hadhsc, Schad, hydroxyacyl-coenzyme A dehydrogenase, mitochondrial, L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain, hydroxylacyl-Coenzyme A dehydrogenase-dehydrogenase, medium and short chain L-3-hydroxyacyl-coenzyme A dehydrogenase, short-chain 3-hydroxyacyl-CoA dehydrogenase
Species: mouse
Products:     Hadh

Top Publications

  1. Nomura M, Takihara Y, Shimada K. Isolation and characterization of retinoic acid-inducible cDNA clones in F9 cells: one of the early inducible clones encodes a novel protein sharing several highly homologous regions with a Drosophila polyhomeotic protein. Differentiation. 1994;57:39-50 pubmed
    ..As the Drosophila polyhomeotic gene is involved in regulating morphogenesis, the rae-28 gene may participate in regulating early mammalian development...
  2. Hardy O, Hohmeier H, Becker T, Manduchi E, Doliba N, Gupta R, et al. Functional genomics of the beta-cell: short-chain 3-hydroxyacyl-coenzyme A dehydrogenase regulates insulin secretion independent of K+ currents. Mol Endocrinol. 2007;21:765-73 pubmed
    ..Among the genes identified was Hadhsc, which encodes short-chain 3-hydroxyacyl-coenzyme A dehydrogenase (SCHAD), an enzyme of mitochondrial beta-..
  3. Li C, Chen P, Palladino A, Narayan S, Russell L, Sayed S, et al. Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase. J Biol Chem. 2010;285:31806-18 pubmed publisher
    ..with hyperinsulinism associated with inactivating mutations of short-chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD) was examined in mice with a knock-out of the hadh gene (hadh(-/-))...
  4. Nomura M, Takihara Y, Shimada K. Isolation of a cDNA clone encoding mouse 3-hydroxyacyl CoA dehydrogenase. Gene. 1995;160:309-10 pubmed
    ..cells, was sequenced, and the deduced RAE-38 protein showed about 86% homology to pig 3-hydroxyacyl CoA dehydrogenase (HCDH; EC This clone can be used to elucidate the regulatory mechanism of HCDH gene expression in mammals.
  5. Tieu K, Perier C, Vila M, Caspersen C, Zhang H, Teismann P, et al. L-3-hydroxyacyl-CoA dehydrogenase II protects in a model of Parkinson's disease. Ann Neurol. 2004;56:51-60 pubmed
    ..Here, we report that L-3-hydroxyacyl-CoA dehydrogenase type II/amyloid binding alcohol dehydrogenase (HADH II/ABAD), a mitochondrial oxidoreductase enzyme involved in neuronal survival, is downregulated in PD patients and ..
  6. Schulz N, Himmelbauer H, Rath M, van Weeghel M, Houten S, Kulik W, et al. Role of medium- and short-chain L-3-hydroxyacyl-CoA dehydrogenase in the regulation of body weight and thermogenesis. Endocrinology. 2011;152:4641-51 pubmed publisher
    ..Medium- and short-chain-3-hydroxyacyl-coenzyme A (CoA) dehydrogenase (SCHAD) (gene name, hadh) catalyze the third reaction of the mitochondrial ?-oxidation cascade, the oxidation of 3-..
  7. Narayan S, Master S, Sireci A, Bierl C, Stanley P, Li C, et al. Short-chain 3-hydroxyacyl-coenzyme A dehydrogenase associates with a protein super-complex integrating multiple metabolic pathways. PLoS ONE. 2012;7:e35048 pubmed publisher
    ..We previously described an interaction between short-chain 3-hydroxyacyl-coenzyme A dehydrogenase (SCHAD) and glutamate dehydrogenase (GDH) explaining the clinical phenotype of hyperinsulinism in SCHAD-deficient ..
  8. Molven A, Hollister Lock J, Hu J, Martinez R, Njølstad P, Liew C, et al. The Hypoglycemic Phenotype Is Islet Cell-Autonomous in Short-Chain Hydroxyacyl-CoA Dehydrogenase-Deficient Mice. Diabetes. 2016;65:1672-8 pubmed publisher
    ..CHI) can be caused by inactivating mutations in the gene encoding short-chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD), a ubiquitously expressed enzyme involved in fatty acid oxidation...
  9. Shekhawat P, Yang H, Bennett M, Carter A, Matern D, Tamai I, et al. Carnitine content and expression of mitochondrial beta-oxidation enzymes in placentas of wild-type (OCTN2(+/+)) and OCTN2 Null (OCTN2(-/-)) Mice. Pediatr Res. 2004;56:323-8 pubmed
    ..at comparable levels in wild-type (OCTN2(+/+)) and homozygous (OCTN2(-/-)) mouse placentas, with the exception of SCHAD, for which activity was significantly higher in OCTN2(-/-) placentas than in OCTN2(+/+) placentas...

More Information


  1. Martin J, Balmer M, Rajendran S, Maurhofer O, Dufour J, St Pierre M. Nutritional stress exacerbates hepatic steatosis induced by deletion of the histidine nucleotide-binding (Hint2) mitochondrial protein. Am J Physiol Gastrointest Liver Physiol. 2016;310:G497-509 pubmed publisher
    ..The absence of Hint2 deregulates the posttranslational modification of several mitochondrial proteins, which impedes the adaptation to episodes of nutritional stress. ..