CPRiL

HADHA

hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha

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]

• trifunctional enzyme subunit alpha, mitochondrial
• 3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit
• 3-oxoacyl-CoA thiolase
78 kDa gastrin-binding protein
gastrin-binding protein
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
long-chain 2-enoyl-CoA hydratase
long-chain-3-hydroxyacyl-CoA dehydrogenase
mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit
mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alpha subunit
mitochondrial trifunctional enzyme, alpha subunit
mitochondrial trifunctional protein, alpha subunit
monolysocardiolipin acyltransferase
ECHA
GBP
HADH
LCEH
LCHAD
MLCL AT
MTPA
TP-ALPHA