Gene Symbol: Hadha
Description: hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha
Alias: RGD1560655, trifunctional enzyme subunit alpha, mitochondrial, TP-alpha, hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit, hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit
Species: rat
Products:     Hadha

Top Publications

  1. Uchida Y, Izai K, Orii T, Hashimoto T. Novel fatty acid beta-oxidation enzymes in rat liver mitochondria. II. Purification and properties of enoyl-coenzyme A (CoA) hydratase/3-hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase trifunctional protein. J Biol Chem. 1992;267:1034-41 pubmed
    ..Therefore, it is concluded that this enzyme is not long-chain 3-hydroxyacyl-CoA dehydrogenase; rather, it is enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase trifunctional protein. ..
  2. Sims H, Brackett J, Powell C, Treem W, Hale D, Bennett M, et al. The molecular basis of pediatric long chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with maternal acute fatty liver of pregnancy. Proc Natl Acad Sci U S A. 1995;92:841-5 pubmed
    ..This is the initial delineation of the molecular basis of isolated LCHAD deficiency. ..
  3. Monstein H, Nylander A, Hakanson R. Widespread tissue expression of gastrin-binding-protein mRNA. Eur J Biochem. 1997;246:502-7 pubmed
    ..The widespread and abundant tissue expression of gastrin-binding-protein mRNA and its sequence identity with a fatty-acid-oxidizing enzyme do not support the view that it represents a genuine gastrin receptor. ..
  4. Lane R, Kelley D, Gruetzmacher E, Devaskar S. Uteroplacental insufficiency alters hepatic fatty acid-metabolizing enzymes in juvenile and adult rats. Am J Physiol Regul Integr Comp Physiol. 2001;280:R183-90 pubmed
    ..These results suggest that uteroplacental insufficiency leads to altered hepatic fatty acid metabolism that may contribute to the adult dyslipidemia associated with low birth weight...
  5. Kamijo T, Aoyama T, Miyazaki J, Hashimoto T. Molecular cloning of the cDNAs for the subunits of rat mitochondrial fatty acid beta-oxidation multienzyme complex. Structural and functional relationships to other mitochondrial and peroxisomal beta-oxidation enzymes. J Biol Chem. 1993;268:26452-60 pubmed
  6. Furuhashi M, Ura N, Murakami H, Hyakukoku M, Yamaguchi K, Higashiura K, et al. Fenofibrate improves insulin sensitivity in connection with intramuscular lipid content, muscle fatty acid-binding protein, and beta-oxidation in skeletal muscle. J Endocrinol. 2002;174:321-9 pubmed
  7. 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
  8. Ibdah J, Perlegas P, Zhao Y, Angdisen J, Borgerink H, Shadoan M, et al. Mice heterozygous for a defect in mitochondrial trifunctional protein develop hepatic steatosis and insulin resistance. Gastroenterology. 2005;128:1381-90 pubmed
    ..Heterozygosity for beta-oxidation defects predisposes to NAFLD and insulin resistance in aging mice. Impairment of mitochondrial beta-oxidation may play an important role in pathogenesis of NAFLD. ..
  9. Asano J, Kudo T, Shimizu T, Fan Y, Nanashima N, Yamana D, et al. Histone acetylation and steroid receptor coactivator expression during clofibrate-induced rat hepatocarcinogenesis. Cancer Sci. 2010;101:869-75 pubmed publisher

More Information


  1. Choi J, Ravipati A, Nimmagadda V, Schubert M, Castellani R, Russell J. Potential roles of PINK1 for increased PGC-1α-mediated mitochondrial fatty acid oxidation and their associations with Alzheimer disease and diabetes. Mitochondrion. 2014;18:41-8 pubmed publisher
  2. Eaton S, Bursby T, Middleton B, Pourfarzam M, Mills K, Johnson A, et al. The mitochondrial trifunctional protein: centre of a beta-oxidation metabolon?. Biochem Soc Trans. 2000;28:177-82 pubmed
    ..We consider the evidence for channelling within the trifunctional protein and propose a model for a beta-oxidation 'metabolon'. ..
  3. Ibdah J, Paul H, Zhao Y, Binford S, Salleng K, Cline M, et al. Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death. J Clin Invest. 2001;107:1403-9 pubmed
    ..Deficiency of MTP causes fetal growth retardation, neonatal hypoglycemia, and sudden death. ..
  4. Liu X, Wu L, Deng G, Li N, Chu X, Guo F, et al. Characterization of mitochondrial trifunctional protein and its inactivation study for medicine development. Biochim Biophys Acta. 2008;1784:1742-9 pubmed publisher
    ..The results indicate that the catalytic residues of the MTP beta-subunit are positioned in the active site similarly to those of monofunctional 3-ketoacyl-CoA thiolase. ..