Synthesis of dicarboxylic acylcarnitines

Syntheses of malonyl, methylmalonyl, succinyl, glutaryl, methylglutaryl, dodecanedioyl and hexadecanedioyl carnitines are described. The dicarboxylic acylcarnitines were prepared from eight equivalents of cyclic anhydride or isopropylidene ester of the dicarboxylic acid and carnitine chloride in trifluoroacetic acid solution. Long chain dicarboxylic acylcarnitines were additionally purified by partitioning between water and n-butanol. Stable isotope labeled analogs, containing 3, 6 or 9 deuterium atoms, were also prepared. They are for use as standards in the electrospray ionization tandem mass spectrometric analysis of dicarboxylic acylcarnitines in samples from patients with inherited disorders of fatty acid oxidation.