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Long-chain acyl-CoA dehydrogenase (EC1.3.8.8, palmitoyl-CoA dehydrogenase, palmitoyl-coenzyme A dehydrogenase, long-chain acyl-coenzyme A dehydrogenase, long-chain-acyl-CoA:(acceptor) 2,3-oxidoreductase, ACADL (gene).) is an enzyme with systematic namelong-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase. This enzyme catalyses the following chemical reaction
a long-chain acyl-CoA + electron-transfer flavoprotein a long-chain trans-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein
This enzyme contains FAD as prosthetic group and participates in fatty acid metabolism and PPAR signaling pathway. Mitochondrial mutations in this enzyme may be associated with some forms of dilated cardiomyopathy.
References
Crane FL, Hauge JG, Beinert H (June 1955). "Flavoproteins involved in the first oxidative step of the fatty acid cycle". Biochimica et Biophysica Acta. 17 (2): 292–4. doi:10.1016/0006-3002(55)90374-7. PMID13239683.
Hall CL, Heijkenskjöld L, Bártfai T, Ernster L, Kamin H (December 1976). "Acyl coenzyme A dehydrogenases and electron-transferring flavoprotein from beef hart mitochondria". Archives of Biochemistry and Biophysics. 177 (2): 402–14. doi:10.1016/0003-9861(76)90453-7. PMID1015826.
Djordjevic S, Dong Y, Paschke R, Frerman FE, Strauss AW, Kim JJ (April 1994). "Identification of the catalytic base in long chain acyl-CoA dehydrogenase". Biochemistry. 33 (14): 4258–64. doi:10.1021/bi00180a021. PMID8155643.