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In vivo neuroprotective effect of L-carnitine against oxidative stress in maple syrup urine disease.
Metab Brain Dis. 2011 Mar; 26(1):21-8.MB

Abstract

Maple syrup urine disease (MSUD) is an autosomal recessive inborn error of metabolism caused by deficiency of the activity of the mitochondrial enzyme complex branched-chain α-keto acid dehydrogenase (BCKAD) leading to accumulation of the branched-chain amino acids (BCAA) leucine, isoleucine and valine and their corresponding branched-chain α-keto acids. Affected patients present severe brain dysfunction manifested such as ataxia, seizures, coma, psychomotor delay and mental retardation. The mechanisms of brain damage in this disease remain poorly understood. Recent studies have shown that oxidative stress may be involved in neuropathology of MSUD. L-Carnitine (L-Car) is considered a potential antioxidant through its action against peroxidation as a scavenger of reactive oxygen species and by its stabilizing effect of damage to cell membranes. In this study we evaluate the possible neuroprotective in vivo effects of L-Car against pro-oxidative effects of BCAA in cerebral cortex of rats. L-Car prevented lipoperoxidation, measured by thiobarbituric acid-reactive substances, protein damage, measured by sulfhydryl and protein carbonyl content and alteration on catalase and glutathione peroxidase activity in rat cortex from a chemically-induced model of MSUD. Our data clearly show that L-Car may be an efficient antioxidant, protecting against the oxidative stress promoted by BCAA. If the present results are confirmed in MSUD patients, this could represent an additional therapeutic approach to the patients affected by this disease.

Authors+Show Affiliations

Programa de Pós-Graduação em Ciências Farmacêuticas, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

21380499

Citation

Mescka, Caroline, et al. "In Vivo Neuroprotective Effect of L-carnitine Against Oxidative Stress in Maple Syrup Urine Disease." Metabolic Brain Disease, vol. 26, no. 1, 2011, pp. 21-8.
Mescka C, Moraes T, Rosa A, et al. In vivo neuroprotective effect of L-carnitine against oxidative stress in maple syrup urine disease. Metab Brain Dis. 2011;26(1):21-8.
Mescka, C., Moraes, T., Rosa, A., Mazzola, P., Piccoli, B., Jacques, C., Dalazen, G., Coelho, J., Cortes, M., Terra, M., Regla Vargas, C., & Dutra-Filho, C. S. (2011). In vivo neuroprotective effect of L-carnitine against oxidative stress in maple syrup urine disease. Metabolic Brain Disease, 26(1), 21-8. https://doi.org/10.1007/s11011-011-9238-x
Mescka C, et al. In Vivo Neuroprotective Effect of L-carnitine Against Oxidative Stress in Maple Syrup Urine Disease. Metab Brain Dis. 2011;26(1):21-8. PubMed PMID: 21380499.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - In vivo neuroprotective effect of L-carnitine against oxidative stress in maple syrup urine disease. AU - Mescka,Caroline, AU - Moraes,Tarsila, AU - Rosa,Andrea, AU - Mazzola,Priscila, AU - Piccoli,Bruna, AU - Jacques,Carlos, AU - Dalazen,Giovana, AU - Coelho,Juliana, AU - Cortes,Marcelo, AU - Terra,Melaine, AU - Regla Vargas,Carmen, AU - Dutra-Filho,Carlos S, Y1 - 2011/03/05/ PY - 2010/12/13/received PY - 2011/02/17/accepted PY - 2011/3/8/entrez PY - 2011/3/8/pubmed PY - 2011/10/1/medline SP - 21 EP - 8 JF - Metabolic brain disease JO - Metab Brain Dis VL - 26 IS - 1 N2 - Maple syrup urine disease (MSUD) is an autosomal recessive inborn error of metabolism caused by deficiency of the activity of the mitochondrial enzyme complex branched-chain α-keto acid dehydrogenase (BCKAD) leading to accumulation of the branched-chain amino acids (BCAA) leucine, isoleucine and valine and their corresponding branched-chain α-keto acids. Affected patients present severe brain dysfunction manifested such as ataxia, seizures, coma, psychomotor delay and mental retardation. The mechanisms of brain damage in this disease remain poorly understood. Recent studies have shown that oxidative stress may be involved in neuropathology of MSUD. L-Carnitine (L-Car) is considered a potential antioxidant through its action against peroxidation as a scavenger of reactive oxygen species and by its stabilizing effect of damage to cell membranes. In this study we evaluate the possible neuroprotective in vivo effects of L-Car against pro-oxidative effects of BCAA in cerebral cortex of rats. L-Car prevented lipoperoxidation, measured by thiobarbituric acid-reactive substances, protein damage, measured by sulfhydryl and protein carbonyl content and alteration on catalase and glutathione peroxidase activity in rat cortex from a chemically-induced model of MSUD. Our data clearly show that L-Car may be an efficient antioxidant, protecting against the oxidative stress promoted by BCAA. If the present results are confirmed in MSUD patients, this could represent an additional therapeutic approach to the patients affected by this disease. SN - 1573-7365 UR - https://www.unboundmedicine.com/medline/citation/21380499/In_vivo_neuroprotective_effect_of_L_carnitine_against_oxidative_stress_in_maple_syrup_urine_disease_ L2 - https://doi.org/10.1007/s11011-011-9238-x DB - PRIME DP - Unbound Medicine ER -