acylkarnitiini

Acylcarnitine refers to a group of molecules derived from carnitine, a quaternary ammonium compound. These molecules are formed when a fatty acid or other acyl group is esterified to the hydroxyl group of carnitine. Acylcarnitines play a crucial role in cellular metabolism, particularly in the transport of activated fatty acids across the inner mitochondrial membrane. This process is essential for beta-oxidation, the breakdown of fatty acids to produce energy in the form of ATP. Different acylcarnitines exist, corresponding to the various acyl groups attached, such as acetyl-CoA, propionyl-CoA, and longer-chain acyl-CoAs. The balance of acylcarnitine levels can be indicative of metabolic status and can be assessed through biochemical testing. Disruptions in acylcarnitine metabolism are associated with various metabolic disorders, including inherited defects in fatty acid oxidation. Measuring specific acylcarnitine profiles is a diagnostic tool used in newborn screening programs for certain metabolic diseases.