Abstract #3808

Carnitine Supplementation in Long-Chain Acyl-CoA Dehydrogenase Knock-Out Mice Is Not Detrimental for Cardiac Function

Adrianus J. Bakermans¹, Michel van Weeghel², Klaas Nicolay¹, Jeanine J. Prompers¹, Sander M. Houten²

¹Biomedical NMR, Eindhoven University of Technology, Eindhoven, Netherlands; ²Laboratory Genetic Metabolic Diseases, Academic Medical Center, Amsterdam, Netherlands

Elevation of long-chain acylcarnitine levels is a hallmark of long-chain fatty acid β-oxidation disorders, and is accompanied by low free carnitine levels. To normalize carnitine concentration, carnitine supplementation has been proposed. However, this could provoke lipotoxic accumulation of long-chain acylcarnitines.