Carnitine O-palmitoyltransferase 2, mitochondrial

Definition:

Involved in the intramitochondrial synthesis of acylcarnitines from accumulated acyl-CoA metabolites. Reconverts acylcarnitines back into the respective acyl-CoA esters that can then undergo beta-oxidation, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. Active with medium (C8-C12) and long-chain (C14-C18) acyl-CoA esters.

References:

[1]. Sara Violante, et al. Carnitine palmitoyltransferase 2: New insights on the substrate specificity and implications for acylcarnitine profiling. Biochim Biophys Acta. 2010 Sep;1802(9):728-32. [Content Brief]

[2]. Tetsuhiko Yasuno, et al. Functional analysis of iPSC-derived myocytes from a patient with carnitine palmitoyltransferase II deficiency. Biochem Biophys Res Commun. 2014 May 30;448(2):175-81. [Content Brief]

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