NCIt definition : Trifunctional enzyme subunit alpha, mitochondrial (763 aa, 83 kDa) is encoded by
the human HADHA gene. This protein is involved in the metabolism of long-chain fatty
acids.;
NCIt note : The mitochondrial trifunctional enzyme is a heterotetramer composed of 2 alpha (HADHA)
and 2 beta (HADHB) subunits.; Trifunctional enzyme subunit alpha, mitochondrial includes two domains. One with long-chain
enoyl-CoA hydratase (EC 4.2.1.17) activity and a second with long chain 3-hydroxyacyl-CoA
dehydrogenase (EC 1.1.1.211) activity; however, this enzyme may also exhibit monolysocardiolipin
acyltransferase (EC 2.3.1.-) activity. (UniProt);