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

Top Publications

  1. Ushikubo S, Aoyama T, Kamijo T, Wanders R, Rinaldo P, Vockley J, et al. Molecular characterization of mitochondrial trifunctional protein deficiency: formation of the enzyme complex is important for stabilization of both alpha- and beta-subunits. Am J Hum Genet. 1996;58:979-88 pubmed
  2. Kao H, Cheng C, Chen Y, Hung S, Huang C, Millington D, et al. ENU mutagenesis identifies mice with cardiac fibrosis and hepatic steatosis caused by a mutation in the mitochondrial trifunctional protein beta-subunit. Hum Mol Genet. 2006;15:3569-77 pubmed
    ..homozygosity mapping with 262 SNP markers mapped the disease gene to chromosome 5 where candidate genes Hadha and Hadhb, encoding the mitochondria trifunctional protein (MTP) alpha- and beta-subunits, respectively, are located...
  3. Frackowiak J, Mazur Kolecka B, Kaczmarski W, Dickson D. Deposition of Alzheimer's vascular amyloid-beta is associated with decreased expression of brain L-3-hydroxyacyl-coenzyme A dehydrogenase (ERAB). Brain Res. 2001;907:44-53 pubmed
    ..Explanation of the association between low levels of HADH and deposition of Abeta by brain smooth muscle cells requires further studies. ..
  4. 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
  5. Seo J, Yaneva R, Hinson E, Cresswell P. Human cytomegalovirus directly induces the antiviral protein viperin to enhance infectivity. Science. 2011;332:1093-7 pubmed publisher
    ..This function of viperin, which was previously attributed to vMIA, suggests that HCMV has coopted viperin to facilitate the infectious process. ..
  6. 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. ..
  7. 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
  8. 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
  9. Choi J, Yoon H, Kim G, Park S, Shin Y, Yoo H. Identification of novel mutations of the HADHA and HADHB genes in patients with mitochondrial trifunctional protein deficiency. Int J Mol Med. 2007;19:81-7 pubmed
    ..Patient 3 was a compound heterozygosity of the HADHB gene, N307D/N389D...

More Information


  1. 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'. ..
  2. 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. ..