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

Abstract

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.

• Authors: Alethéa G Barschak, Angela Sitta, Marion Deon, Estela N B Busanello, Daniella M Coelho, Franciele Cipriani, Carlos S Dutra-Filho, Roberto Giugliani, Moacir Wajner, Carmen R Vargas
• Journal: Clinical biochemistry (Clin Biochem) Vol. 42 Issue 6 Pg. 462-6 (Apr 2009) ISSN: 1873-2933 [Electronic] United States
• PMID: 19121297 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Amino Acids
• Antioxidants
• Hydroxy Acids
• Keto Acids
• Thiobarbituric Acid Reactive Substances
• Isoleucine
• Tryptophan
• Methionine
• Leucine
• Valine

Topics

• Adult
• Amino Acids (blood)
• Antioxidants (metabolism)
• Humans
• Hydroxy Acids (blood)
• Isoleucine (blood)
• Keto Acids (blood)
• Leucine (blood)
• Lipid Peroxidation (physiology)
• Maple Syrup Urine Disease (blood)
• Methionine (blood)
• Oxidative Stress
• Thiobarbituric Acid Reactive Substances (metabolism)
• Tryptophan (blood)
• Valine (blood)