QUANTIFICATION OF FREE CARNITINE, INDIVIDUAL SHORT-CHAIN AND MEDIUM-CHAIN ACYLCARNITINES, AND TOTAL CARNITINE IN PLASMA BY HIGH-PERFORMANCE LIQUID-CHROMATOGRAPHY

This paper describes a method for the quantitative determination of free carnitine, acetylcarnitine, propionylcarnitine, hexanoylcarnitine, octanoylcarnitine, and total carnitine in plasma. Carnitine and acylcarnitines were extracted from 100 μl of plasma with acetonitrile/methanol and isolated using 0.5-ml columns of silica gel. Samples were then derivatized with 4′-broniophenacyl trifluoromethanesulfonate and quantified by high-performance liquid chromatography with detection at 260 nm. Carnitine and acylcarnitines were quantified in normal human plasma and the plasma of patients diagnosed with methylmalonic aciduria, propionic acidemia, and medium-chain acyl-CoA dehydrogenase deficiency. © 1993 Academic Press, Inc.