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Official Full Name
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit
This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. [provided by RefSeq, Jul 2008]
HADHA; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA; trifunctional enzyme subunit alpha, mitochondrial; 3-oxoacyl-CoA thiolase; gastrin-binding protein; 78 kDa gastrin-binding protein; long-chain 2-enoyl-CoA hydratase; long-chain-3-hydroxyacyl-CoA dehydrogenase; mitochondrial trifunctional enzyme, alpha subunit; mitochondrial trifunctional protein, alpha subunit; 3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit; mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit; mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alpha subunit; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit; hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein), alpha subunit; gastrin binding protein; long chain 2 enoyl CoA hydratase; long chain 3 hydroxyacyl CoA dehydrogenase; mitochondrial trifunctional protein; alpha subunit; MGC1728; GBP, ECHA, HADH, LCEH, MTPA, LCHAD, TP-ALPHA; CFR-associated protein p70; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzym