| Function | Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity. Independently of subunit beta, HADHA also exhibits a cardiolipin acyltransferase activity that participates in cardiolipin remodeling.cardiolipin is a major mitochondrial membrane phospholipid. HADHA may act downstream of Tafazzin/TAZ, that remodels monolysocardiolipin (MLCL) to a cardiolipin intermediate, and then HADHA may continue to remodel this species into mature tetralinoleoyl-cardiolipin. Has also been proposed to act directly on MLCL.capable of acylating MLCL using different acyl-CoA substrates, with highest activity for oleoyl-CoA. |
| Protein Name | Trifunctional Enzyme Subunit Alpha - Mitochondrial78 Kda Gastrin-Binding ProteinMonolysocardiolipin AcyltransferaseMlcl AtTp-Alpha Includes - Long-Chain Enoyl-Coa Hydratase - Long Chain 3-Hydroxyacyl-Coa Dehydrogenase |
| Database Links | Reactome: R-HSA-1482798Reactome: R-HSA-77285Reactome: R-HSA-77288Reactome: R-HSA-77305Reactome: R-HSA-77310Reactome: R-HSA-77346Reactome: R-HSA-77348Reactome: R-HSA-77350 |
| Cellular Localisation | MitochondrionMitochondrion Inner MembraneProtein Stability And Association With Mitochondrion Inner Membrane Do Not Require Hadhb |
| Alternative Antibody Names | Anti-Trifunctional Enzyme Subunit Alpha - Mitochondrial antibodyAnti-78 Kda Gastrin-Binding Protein antibodyAnti-Monolysocardiolipin Acyltransferase antibodyAnti-Mlcl At antibodyAnti-Tp-Alpha Includes - Long-Chain Enoyl-Coa Hydratase - Long Chain 3-Hydroxyacyl-Coa Dehydrogenase antibodyAnti-HADHA antibodyAnti-HADH antibody |
Information sourced from Uniprot.org
