L-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders

doi:10.1016/j.gene.2013.10.017

PMID: 24148561DOI: 10.1016/j.gene.2013.10.017Oct 24, 2013Paper

L-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders

Gene

Graziela S RibasMoacir Wajner

Abstract

In recent years increasing evidence has emerged suggesting that oxidative stress is involved in the pathophysiology of a number of inherited metabolic disorders. However the clinical use of classical antioxidants in these diseases has been poorly evaluated and so far no benefit has been demonstrated. l-Carnitine is an endogenous substance that acts as a carrier for fatty acids across the inner mitochondrial membrane necessary for subsequent beta-oxidation and ATP production. Besides its important role in the metabolism of lipids, l-carnitine is also a potent antioxidant (free radical scavenger) and thus may protect tissues from oxidative damage. This review addresses recent findings obtained from patients with some inherited neurometabolic diseases showing that l-carnitine may be involved in the reduction of oxidative damage observed in these disorders. For some of these diseases, reduced concentrations of l-carnitine may occur due to the combination of this compound to the accumulating toxic metabolites, especially organic acids, or as a result of protein restricted diets. Thus, l-carnitine supplementation may be useful not only to prevent tissue deficiency of this element, but also to avoid oxidative damage secondary to incre...Continue Reading

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