ACYLCARNITINES IN INTERMEDIARY METABOLISM

From the time of its discovery in 1905 until the first description of its deficiency in 1973, the role of carnitine in intermediary metaboli sm was decidedly vague. Identification of carnitine acyl transferases and their products, acylcarnitines, have paved the way to the confirma tion of the importance of carnitine in the transfer of fatty acid CoAs into the mitochondrion for beta-oxidatian and energy production. The elucidation of defects in fatty acid oxidation together with the conce pt of carnitine therapy in certain organoacidaemias have given a new m eaning to the term acylcarnitine. Not only are these compounds of diag nostic importance, their formation may be part of a secondary carnitin e depletion which may be brought about as a result of various medicati ons. Recent evidence suggests that long-chain acylcarnitines are respo nsible for cardiac arrhythmias and other effects? both good and bad, w ill certainly be found. This review will attempt to highlight the impo rtance of acylcarnitines, from their production, the difficulties in a nalysis, the diagnostic possibilities and their positive and negative effects on intermediary metabolism.