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The defect in the branched\u2010chain \u03b1\u2010keto acid dehydrogenase complex activity leads to an accumulation of these compounds and their corresponding \u03b1\u2010keto\u2010acids and \u03b1\u2010hydroxy\u2010acids. 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), which has demonstrated an important role as antioxidant by reducing and scavenging free radicals formation and by enhancing the activity of antioxidant enzymes, have been used in the treatment of some metabolic rare disorders. This study evaluated the oxidative stress parameters, di\u2010tyrosine, isoprostanes and antioxidant capacity, in urine of MSUD patients under protein\u2010restricted diet supplemented or not with <jats:sc>l<\/jats:sc>\u2010car capsules at a dose of 50 mg kg<jats:sup>\u22121<\/jats:sup> day<jats:sup>\u22121<\/jats:sup>. It was also determined urinary \u03b1\u2010keto isocaproic acid levels as well as blood free <jats:sc>l<\/jats:sc>\u2010car concentrations in blood. It was found a deficiency of carnitine in patients before the <jats:sc>l<\/jats:sc>\u2010car supplementation. Significant increases of di\u2010tyrosine and isoprostanes, as well as reduced antioxidant capacity, were observed before the treatment with <jats:sc>l<\/jats:sc>\u2010car. The <jats:sc>l<\/jats:sc>\u2010car supplementation induced beneficial effects on these parameters reducing the di\u2010tyrosine and isoprostanes levels and increasing the antioxidant capacity. It was also showed a significant increase in urinary of \u03b1\u2010ketoisocaproic acid after 2 months of <jats:sc>l<\/jats:sc>\u2010car treatment, compared to control group. In conclusion, our results suggest that <jats:sc>l<\/jats:sc>\u2010car may have beneficial effects in the treatment of MSUD by preventing oxidative damage to the cells and that urine can be used to monitorize oxidative damage in patients affected by this disease.<\/jats:p>","DOI":"10.1016\/j.ijdevneu.2015.02.003","type":"journal-article","created":{"date-parts":[[2015,2,10]],"date-time":"2015-02-10T07:21:08Z","timestamp":1423552868000},"page":"10-14","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["Urinary biomarkers of oxidative damage in Maple syrup urine disease: The <scp>l<\/scp>\u2010carnitine role"],"prefix":"10.1002","volume":"42","author":[{"given":"Gilian","family":"Guerreiro","sequence":"first","affiliation":[{"name":"Faculdade de Farm\u00e1cia UFRGS Av. 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