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HADHA
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 the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity. 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. Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity. In the central section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
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| Protein type: Acetyltransferase; Amino Acid Metabolism - lysine degradation; Amino Acid Metabolism - tryptophan; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - butanoate; Carbohydrate Metabolism - propanoate; EC 1.1.1.211; EC 4.2.1.17; Lipid Metabolism - fatty acid; Lipid Metabolism - fatty acid elongation in mitochondria; Lipid Metabolism - unsaturated fatty acid biosynthesis; Lyase; Mitochondrial; Other Amino Acids Metabolism - beta-alanine; Oxidoreductase; Secondary Metabolites Metabolism - limonene and pinene degradation |
| Chromosomal Location of mouse Ortholog: 5|5 B1 |
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Cellular Component:
membrane; mitochondrial fatty acid beta-oxidation multienzyme complex; mitochondrial inner membrane; mitochondrial nucleoid; mitochondrion
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Molecular Function:
3-hydroxyacyl-CoA dehydratase activity; 3-hydroxyacyl-CoA dehydrogenase activity; acetyl-CoA C-acyltransferase activity; catalytic activity; enoyl-CoA hydratase activity; fatty-acyl-CoA binding; long-chain-3-hydroxyacyl-CoA dehydrogenase activity; lyase activity; NAD binding; NAD+ binding; oxidoreductase activity; oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor; protein binding; protein-containing complex binding; transferase activity
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Biological Process:
cardiolipin acyl-chain remodeling; fatty acid beta-oxidation; fatty acid metabolic process; lipid metabolic process; metabolic process; response to insulin; response to xenobiotic stimulus
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Reference #:
Q8BMS1
(UniProtKB)
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Alt. Names/Synonyms: C77020; ECHA; Hadha; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit; Long chain 3-hydroxyacyl-CoA dehydrogenase; Long-chain enoyl-CoA hydratase; Monolysocardiolipin acyltransferase; Mtpa; OTTMUSP00000029769; TP-alpha; Trifunctional enzyme subunit alpha, mitochondrial
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Gene Symbols: Hadha
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Molecular weight:
82,670 Da
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Basal Isoelectric point:
9.24
Predict pI for various phosphorylation states
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