HADHA (Vector pUC, Accession No. BC009235)

Length: 2292 Sequence: atggtggcct gccgggcgat tggcatcctc agccgctttt ctgccttcag gatcctccgc tcccgaggtt atatatgccg caattttaca gggtcttctg ctttgctgac cagaacccat attaactatg gagtcaaagg ggatgtggca gttgttcgaa ttaactctcc caattcaaag gtaaatacac tgagtaaaga gctacattca gagttctcag aagttatgaa tgaaatctgg gctagtgatc aaatcagaag tgccgtcctt atctcatcaa agccaggctg ctttattgca ggtgctgata tcaacatgtt agccgcttgc aagacccttc aagaagtaac acagctatca caagaagcac agagaatagt tgagaaactt gaaaagtcca caaagcctat tgtggctgcc atcaatggat cctgcctggg aggaggactt gaggttgcca tttcatgcca atacagaata gcaacaaaag acagaaaaac agtattaggt acccctgaag ttttgctggg ggccttacca ggagcaggag gcacacaaag gctgcccaaa atggtgggtg tgcctgctgc tttggacatg atgctgactg gtagaagcat tcgtgcagac agggcaaaga aaatgggact ggttgaccaa ctggtggaac ccctgggacc aggactaaaa cctccagagg aacggacaat agaataccta gaagaagttg caattacttt tgccaaagga ctagctgata agaagatctc tccaaagaga gacaagggat tggtggaaaa attgacagcg tatgccatga ctattccatt tgtcaggcaa caggtttaca aaaaagtgga agaaaaagtg cgaaagcaga ctaaaggcct ttatcctgca cctctgaaaa taattgatgt ggtaaagact ggaattgagc aagggagtga tgccggttat ctctgtgaat ctcagaaatt tggagagctt gtaatgacca aagaatcaaa ggccttgatg ggactctacc atggtcaggt cctgtgcaag aagaataaat ttggagctcc acagaaggat gttaagcatc tggctattct tggtgcaggg ctgatgggag caggcatcgc ccaagtctcc gtggataagg ggctaaagac tatacttaaa gatgccaccc tcactgcgct agaccgagga cagcaacaag tgttcaaagg attgaatgac aaagtgaaga agaaagctct aacatcattt gaaagggatt ccatcttcag caacttgact gggcagcttg attaccaagg ttttgaaaag gccgacatgg tgattgaagc tgtgtttgag gaccttagtc ttaagcacag agtgctaaag gaagtagaag cggtgattcc agatcactgt atctttgcca gtaacacatc tgctctccca atcagtgaaa tcgctgctgt cagcaaaaga cctgagaagg tgattggcat gcactacttc tctcccgtgg acaagatgca gctgctggag attatcacga ccgagaaaac ttccaaagac accagtgctt cagctgtagc agttggtctc aagcagggga aggtcatcat tgtggttaag gatggacctg gcttctatac taccaggtgt cttgcgccca tgatgtctga agtcatccga atcctccagg aaggagttga cccgaagaag ctggattccc tgaccacaag ctttggcttt cctgtgggtg ccgccacact ggtggatgaa gttggtgtgg atgtagcgaa acatgtggcg gaagatctgg gcaaagtctt tggggagcgg tttggaggtg gaaacccaga actgctgaca cagatggtgt ccaagggctt cctaggtcgt aaatctggga agggctttta catctatcag gagggtgtga agaggaagga tttgaattct gacatggata gtattttagc gagtctgaag ctgcctccta agtctgaagt ctcatcagac gaagacatcc agttccgcct ggtgacaaga tttgtgaatg aggcagtcat gtgcctgcaa gaggggatct tggccacacc tgcagaggga gacatcggag ccgtctttgg gcttggcttc ccgccttgtc tgggagggcc tttccgcttt gtggatctgt atggcgccca gaagatagtg gaccggctca agaaatatga agctgcctat ggaaaacagt tcaccccatg ccagctgcta gctgaccatg ctaacagccc taacaagaag ttctaccagt Protein 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). [The UniProt Consortium]