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This disorder is clinically characterized by ketoacidosis, seizures, coma, psychomotor delay and mental retardation whose pathophysiology is not completely understood. Recent studies have shown that oxidative stress may be involved in neuropathology of MSUD. <jats:sc>l<\/jats:sc>\u2010Carnitine (<jats:sc>l<\/jats:sc>\u2010Car) plays a central role in the cellular energy metabolism because it transports long\u2010chain fatty acids for oxidation and ATP generation. In recent years many studies have demonstrated the antioxidant role of this compound. In this work, we investigated the effect of BCAA\u2010restricted diet supplemented or not with <jats:sc>l<\/jats:sc>\u2010Car on lipid peroxidation and in protein oxidation in MSUD patients. We found a significant increase of malondialdehyde and of carbonyl content in plasma of MSUD patients under BCAA\u2010restricted diet compared to controls. Furthermore, patients under BCAA\u2010restricted diet plus <jats:sc>l<\/jats:sc>\u2010Car supplementation presented a marked reduction of malondialdehyde content in relation to controls, reducing the lipid peroxidation. In addition, free <jats:sc>l<\/jats:sc>\u2010Car concentrations were negatively correlated with malondialdehyde levels. Our data show that <jats:sc>l<\/jats:sc>\u2010Car may have an antioxidant effect, protecting against the lipid peroxidation and this could represent an additional therapeutic approach to the patients affected by MSUD.<\/jats:p>","DOI":"10.1016\/j.ijdevneu.2012.10.109","type":"journal-article","created":{"date-parts":[[2012,11,5]],"date-time":"2012-11-05T23:32:26Z","timestamp":1352158346000},"page":"21-24","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":45,"title":["Protein and lipid damage in maple syrup urine disease patients: <scp>l<\/scp>\u2010carnitine effect"],"prefix":"10.1002","volume":"31","author":[{"given":"Caroline Paula","family":"Mescka","sequence":"first","affiliation":[{"name":"Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em CB:Bioqu\u00edmica e Departamento de Bioqu\u00edmica UFRGS Rua Ramiro Barcelos, 2600 90035\u2010000 Porto Alegre RS Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos Alberto Yasin","family":"Wayhs","sequence":"additional","affiliation":[{"name":"Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Ci\u00eancias Farmac\u00eauticas UFRGS Av. 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