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Amino acids levels and lipid peroxidation in maple syrup urine disease patients.
Clin Biochem. 2009 Apr; 42(6):462-6.CB

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+Show Affiliations

Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil. aletheagatto@gmail.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

19121297

Citation

Barschak, Alethéa G., et al. "Amino Acids Levels and Lipid Peroxidation in Maple Syrup Urine Disease Patients." Clinical Biochemistry, vol. 42, no. 6, 2009, pp. 462-6.
Barschak AG, Sitta A, Deon M, et al. Amino acids levels and lipid peroxidation in maple syrup urine disease patients. Clin Biochem. 2009;42(6):462-6.
Barschak, A. G., Sitta, A., Deon, M., Busanello, E. N., Coelho, D. M., Cipriani, F., Dutra-Filho, C. S., Giugliani, R., Wajner, M., & Vargas, C. R. (2009). Amino acids levels and lipid peroxidation in maple syrup urine disease patients. Clinical Biochemistry, 42(6), 462-6. https://doi.org/10.1016/j.clinbiochem.2008.12.005
Barschak AG, et al. Amino Acids Levels and Lipid Peroxidation in Maple Syrup Urine Disease Patients. Clin Biochem. 2009;42(6):462-6. PubMed PMID: 19121297.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Amino acids levels and lipid peroxidation in maple syrup urine disease patients. AU - Barschak,Alethéa G, AU - Sitta,Angela, AU - Deon,Marion, AU - Busanello,Estela N B, AU - Coelho,Daniella M, AU - Cipriani,Franciele, AU - Dutra-Filho,Carlos S, AU - Giugliani,Roberto, AU - Wajner,Moacir, AU - Vargas,Carmen R, Y1 - 2008/12/24/ PY - 2008/09/02/received PY - 2008/11/26/revised PY - 2008/12/03/accepted PY - 2009/1/6/entrez PY - 2009/1/6/pubmed PY - 2009/6/2/medline SP - 462 EP - 6 JF - Clinical biochemistry JO - Clin Biochem VL - 42 IS - 6 N2 - 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. SN - 1873-2933 UR - https://www.unboundmedicine.com/medline/citation/19121297/Amino_acids_levels_and_lipid_peroxidation_in_maple_syrup_urine_disease_patients_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0009-9120(08)00624-3 DB - PRIME DP - Unbound Medicine ER -