FUNCTION: Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (PubMed:8135828, PubMed:1550553, PubMed:29915090, PubMed:30850536). The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is...
FUNCTION: Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (PubMed:8135828, PubMed:1550553, PubMed:29915090, PubMed:30850536). 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 (PubMed:29915090). Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids (PubMed:30850536). 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 (PubMed:8135828, PubMed:29915090, PubMed:30850536). Independently of the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity (PubMed:23152787). It acylates monolysocardiolipin into cardiolipin, a major mitochondrial membrane phospholipid which plays a key role in apoptosis and supports mitochondrial respiratory chain complexes in the generation of ATP (PubMed:23152787). Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity (PubMed:23152787). {ECO:0000269|PubMed:1550553, ECO:0000269|PubMed:23152787, ECO:0000269|PubMed:29915090, ECO:0000269|PubMed:30850536, ECO:0000269|PubMed:8135828, ECO:0000303|PubMed:29915090, ECO:0000303|PubMed:30850536}.
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GO - Biological processes (BP):
cardiolipin acyl-chain remodeling [GO:0035965]; fatty acid beta-oxidation [GO:0006635]; response to insulin [GO:0032868]; response to xenobiotic stimulus [GO:0009410]...
cardiolipin acyl-chain remodeling [GO:0035965]; fatty acid beta-oxidation [GO:0006635]; response to insulin [GO:0032868]; response to xenobiotic stimulus [GO:0009410]
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GO - Molecular function (MF):
3-hydroxyacyl-CoA dehydrogenase activity [GO:0003857]; acetyl-CoA C-acetyltransferase activity [GO:0003985]; enoyl-CoA hydratase activity [GO:0004300]; fatty-acyl-CoA binding [GO:0000062]; long-chain-3-hydroxyacyl-CoA dehydrogenase activity [GO:0016509]; long-chain-enoyl-CoA hydratase activity [GO:0...
3-hydroxyacyl-CoA dehydrogenase activity [GO:0003857]; acetyl-CoA C-acetyltransferase activity [GO:0003985]; enoyl-CoA hydratase activity [GO:0004300]; fatty-acyl-CoA binding [GO:0000062]; long-chain-3-hydroxyacyl-CoA dehydrogenase activity [GO:0016509]; long-chain-enoyl-CoA hydratase activity [GO:0016508]; NAD+ binding [GO:0070403]; protein-containing complex binding [GO:0044877]
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GO - Cellular component (CC):
mitochondrial fatty acid beta-oxidation multienzyme complex [GO:0016507]; mitochondrial inner membrane [GO:0005743]; mitochondrial nucleoid [GO:0042645]; mitochondrion [GO:0005739]...
mitochondrial fatty acid beta-oxidation multienzyme complex [GO:0016507]; mitochondrial inner membrane [GO:0005743]; mitochondrial nucleoid [GO:0042645]; mitochondrion [GO:0005739]
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