| In vivo neuroprotective effect of L-carnitine against oxidative stress in maple syrup urine disease. | |
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MedLine Citation:
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PMID: 21380499 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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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. |
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Authors:
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Caroline Mescka; Tarsila Moraes; Andrea Rosa; Priscila Mazzola; Bruna Piccoli; Carlos Jacques; Giovana Dalazen; Juliana Coelho; Marcelo Cortes; Melaine Terra; Carmen Regla Vargas; Carlos S Dutra-Filho |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-3-5 |
Journal Detail:
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Title: Metabolic brain disease Volume: - ISSN: 1573-7365 ISO Abbreviation: - Publication Date: 2011 Mar |
Date Detail:
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Created Date: 2011-3-7 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8610370 Medline TA: Metab Brain Dis Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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Programa de Pós-Graduação em Ciências Farmacêuticas, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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