Objective: In the present study we correlated the amino acids, branched-chain alpha-keto acids and alpha-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 methods: TBARS, amino acids, branched-chain alpha-keto acids and alpha-hydroxy acids concentrations were measured in plasma samples from treated MSUD patients.
Results: We 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.
Conclusions: Considering 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.