Gene Symbol: Hadha
Description: hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit
Alias: C77020, Mtpa, trifunctional enzyme subunit alpha, mitochondrial, TP-alpha
Species: mouse
Products:     Hadha

Top Publications

  1. 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
    ..The Mtpa(-/-) fetuses accumulate long chain fatty acid metabolites and have low birth weight compared with the Mtpa(+/-) and ..
  2. 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
  3. 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
    ..Recently, we generated a mouse model for MTP deficiency and reported that homozygous (MTPa-/-) mice suffer neonatal death...
  4. Kao Y, Chang B, Liang J, Tsai H, Lee Y, Lin R, et al. Japanese encephalitis virus nonstructural protein NS5 interacts with mitochondrial trifunctional protein and impairs fatty acid β-oxidation. PLoS Pathog. 2015;11:e1004750 pubmed publisher
    ..We identified a novel function of JEV NS5 in viral pathogenesis by impairing LCFA β-oxidation and inducing cytokine expression by association with MTP. ..
  5. Mejia E, Ibdah J, Sparagna G, Hatch G. Differential reduction in cardiac and liver monolysocardiolipin acyltransferase-1 and reduction in cardiac and liver tetralinoleoyl-cardiolipin in the α-subunit of trifunctional protein heterozygous knockout mice. Biochem J. 2015;471:123-9 pubmed publisher
    ..formation was examined in heart and liver mitochondria from wild-type (WT) and αTFP heterozygous knockout [Mtpa(+/-)] mice...
  6. Maeyashiki C, Oshima S, Otsubo K, Kobayashi M, Nibe Y, Matsuzawa Y, et al. HADHA, the alpha subunit of the mitochondrial trifunctional protein, is involved in long-chain fatty acid-induced autophagy in intestinal epithelial cells. Biochem Biophys Res Commun. 2017;484:636-641 pubmed publisher
    ..b>HADHA was identified as an LC3-interacting protein from primary IECs...