Carnitine supplementation attenuates myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout mice

@article{Bakermans2013CarnitineSA,
  title={Carnitine supplementation attenuates myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout mice},
  author={Adrianus J. Bakermans and Michel van Weeghel and Simone W Denis and Klaas Nicolay and J. J. Prompers and Sander M. Houten},
  journal={Journal of Inherited Metabolic Disease},
  year={2013},
  volume={36},
  pages={973-981}
}
Elevation of long-chain acylcarnitine levels is a hallmark of long-chain mitochondrial β-oxidation (FAO) disorders, and can be accompanied by secondary carnitine deficiency. To restore free carnitine levels, and to increase myocardial export of long-chain fatty acyl-CoA esters, supplementation of L-carnitine in patients has been proposed. However, carnitine supplementation is controversial, because it may enhance the potentially lipotoxic buildup of long-chain acylcarnitines in the FAO… CONTINUE READING