Peroxisomal carnitine O-octanoyltransferase is an enzyme that in humans is encoded by the CROTgene.[1][2]
Carnitine octanoyltransferase (EC 2.3.1.137) is a carnitine acyltransferase that catalyzes the reversible transfer of fatty acyl groups between CoA and carnitine. This enzyme regulates the breakdown of Very Long Chain Fatty Acids (VLCFA) by performing a crucial step in the transport of medium length acyl chains out of the mammalian peroxisome to the cytosol and mitochondria for further breakdown. See also CRAT (MIM 600184). Van der Leij et al. (2000) reviewed the function, structural features, and phylogenetics of human carnitine acyltransferase genes, including CROT.[supplied by OMIM][2]
References
↑Ferdinandusse S, Mulders J, IJlst L, Denis S, Dacremont G, Waterham HR, Wanders RJ (Oct 1999). "Molecular cloning and expression of human carnitine octanoyltransferase: evidence for its role in the peroxisomal beta-oxidation of branched-chain fatty acids". Biochem Biophys Res Commun. 263 (1): 213–8. doi:10.1006/bbrc.1999.1340. PMID10486279.
van der Leij FR, Huijkman NC, Boomsma C, et al. (2000). "Genomics of the human carnitine acyltransferase genes". Mol. Genet. Metab. 71 (1–2): 139–53. doi:10.1006/mgme.2000.3055. PMID11001805.
Morillas M, Gómez-Puertas P, Rubí B, et al. (2002). "Structural model of a malonyl-CoA-binding site of carnitine octanoyltransferase and carnitine palmitoyltransferase I: mutational analysis of a malonyl-CoA affinity domain". J. Biol. Chem. 277 (13): 11473–80. doi:10.1074/jbc.M111628200. PMID11790793.
