Amino acids levels and lipid peroxidation in maple syrup urine disease patients

ObjectiveIn the present study we correlated the amino acids, branched-chain α-keto acids and α-hydroxy acids levels with the thiobarbituric acid-reactive species (TBARS) measurement, a lipid peroxidation parameter, in plasma from treated MSUD patients in order to examine whether these accumulated metabolites could be associated to the oxidative stress present in MSUD.Design and methodsTBARS, amino acids, branched-chain α-keto acids and α-hydroxy acids concentrations were measured in plasma samples from treated MSUD patients.ResultsWe verified that plasma TBARS was increased, whereas tryptophan and methionine concentrations were significantly reduced. Furthermore TBARS measurement was inversely correlated to methionine and tryptophan levels.ConclusionsConsidering that methionine and tryptophan have antioxidant activities, the data suggest that the imbalance of these amino acids may be involved with lipid peroxidation in MSUD.