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
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 ..
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
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...
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 Î²-oxidation. PLoS Pathog. 2015;11:e1004750 pubmed publisher
..We identified a novel function of JEV NS5 in viral pathogenesis by impairing LCFA Î²-oxidation and inducing cytokine expression by association with MTP. ..
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 Î±-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 Î±TFP heterozygous knockout [Mtpa(+/-)] mice...
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...