Determination of the reference range of endogenous plasma carnitines in healthy adults

摘要

Disturbances in carnitine homeostasis have been shown to impact greatly on human health. L-carnitine is an endogenous substance present in all mammalian species covering a wide range of biological activities. In addition to its role in the coenzyme A metabolism and as scavenger for unwanted acyl groups, the most important role is the transport of fatty acids across the inner mitochondrial membrane for subsequent beta-oxidation, the fundamental source of cellular energy. Several metabolic as well as secondary disorders (e. g. drug-induced by pivampicillin or valproic acid) associated with low carnitine levels may lead to cardiomyopathy. Encephalopathy, muscle weakness and even early death when untreated. Despite the fact that plasma levels represents only about 1% of the total body carnitine pool, it is commonly used as an indicator of the total body carnitine status.