Octanoyl-DL-carnitine (chloride)

Carnitine facilitates the transport of acyl units across the inner mitochondrial membrane. Fatty acid esters of carnitine (acylcarnitines) have been shown to inhibit the oxidation of long chain fatty acids by interfering with the mitochondrial carnitine:acylcarnitine exchange system. Octanoyl-DL-carnitine is a carnitine ester that inhibits carnitine-acylcarnitine translocase with an IC50 value of 33 μM. Medium-chain acyl-CoA dehydrogenase deficiency, a risk factor for sudden unexpected infant death, leads to the accumulation of octanoyl-DL-carnitine in the blood. Thus, analysis of neonatal blood concentrations of octanoyl-DL-carnitine by tandem mass spectrometry has been used as part of a standard newborn screening program as a biomarker of this deficiency.