They are synthesized by the enzyme carnitine palmitoyltransferase 1 (CPT 1) that is known to be responsible for the transport of fatty acids into the mitochondrial matrix.[24] Our analysis revealed significant differences in the concentrations of the acylcarnitine, dodecanoylcarnitine, which is present in fatty acid oxidation disorders such as long-chain acyl CoA dehydrogenase deficiency, carnitine palmitoyltransferase I deficiency, and carnitine palmitoyltransferase II deficiency. The gene discussed is CPT1C; the disease is hyperinsulinemic hypoglycemia, familial, 4.