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L-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders.
Gene. 2014 Jan 10; 533(2):469-76.GENE

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 increased production of reactive species in these diseases. Considering the ability of l-carnitine to easily cross the blood-brain barrier, l-carnitine supplementation may also be beneficial in preventing neurological damage derived from oxidative injury. However further studies are required to better explore this potential.

Authors+Show Affiliations

Federal University of Rio Grande do Sul, Brazil; Serviço de Genética Médica, HCPA, Ramiro Barcelos 2350, Porto Alegre, RS 90035-903, Brazil.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

24148561

Citation

Ribas, Graziela S., et al. "L-carnitine Supplementation as a Potential Antioxidant Therapy for Inherited Neurometabolic Disorders." Gene, vol. 533, no. 2, 2014, pp. 469-76.
Ribas GS, Vargas CR, Wajner M. L-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders. Gene. 2014;533(2):469-76.
Ribas, G. S., Vargas, C. R., & Wajner, M. (2014). L-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders. Gene, 533(2), 469-76. https://doi.org/10.1016/j.gene.2013.10.017
Ribas GS, Vargas CR, Wajner M. L-carnitine Supplementation as a Potential Antioxidant Therapy for Inherited Neurometabolic Disorders. Gene. 2014 Jan 10;533(2):469-76. PubMed PMID: 24148561.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - L-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders. AU - Ribas,Graziela S, AU - Vargas,Carmen R, AU - Wajner,Moacir, Y1 - 2013/10/19/ PY - 2013/08/23/received PY - 2013/10/02/revised PY - 2013/10/09/accepted PY - 2013/10/24/entrez PY - 2013/10/24/pubmed PY - 2014/1/24/medline KW - 4-hydroxy-2-nonenal KW - ALC KW - ATP KW - Antioxidants KW - BCAA KW - BCKAD KW - CAT KW - CNS KW - CPT KW - GPx KW - GSH KW - H(2)O(2) KW - HD KW - HNE KW - HSP KW - Huntington's disease KW - LC KW - LCAD KW - LCHAD KW - MCAD KW - MDA KW - MSUD KW - Neurometabolic disorders KW - Oxidative stress KW - PI3K KW - PKU KW - PLC KW - ROS KW - SOD KW - TAR KW - TBARS KW - VLCAD KW - acetyl-l-carnitine KW - adenosine triphosphate KW - branched-chain amino acids KW - branched-chain α-keto acid dehydrogenase complex KW - carnitine palmitoyltransferase KW - catalase KW - central nervous system KW - glutathione peroxidase KW - heat shock protein KW - hydrogen peroxide KW - l-Carnitine KW - long-chain 3-hydroxyacyl-CoA dehydrogenase KW - long-chain acyl-CoA dehydrogenase KW - malondialdehyde KW - maple syrup urine disease KW - medium-chain acyl-coenzyme A dehydrogenase KW - phenylketonuria KW - phosphoinositol-3 kinase KW - propionyl-l-carnitine KW - reactive oxygen species KW - reduced glutathione KW - superoxide dismutase KW - thiobarbituric acid reactive species KW - total antioxidant reactivity KW - very-long-chain acyl-CoA dehydrogenase SP - 469 EP - 76 JF - Gene JO - Gene VL - 533 IS - 2 N2 - 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 increased production of reactive species in these diseases. Considering the ability of l-carnitine to easily cross the blood-brain barrier, l-carnitine supplementation may also be beneficial in preventing neurological damage derived from oxidative injury. However further studies are required to better explore this potential. SN - 1879-0038 UR - https://www.unboundmedicine.com/medline/citation/24148561/L_carnitine_supplementation_as_a_potential_antioxidant_therapy_for_inherited_neurometabolic_disorders_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0378-1119(13)01411-X DB - PRIME DP - Unbound Medicine ER -