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Oxidative stress in plasma from maple syrup urine disease patients during treatment

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Abstract

Maple Syrup Urine Disease (MSUD) is an autossomal recessive metabolic disorder caused by a deficiency of branched-chain α-keto acid dehydrogenase complex activity leading to accumulation of the branched-chain amino acids leucine, isoleucine and valine and their corresponding branched-chain α-keto acids. Affected patients usually present hypoglycemia, ketoacidosis, convulsions, poor feeding, coma, psychomotor delay and mental retardation. Considering that the pathophysiology of MSUD is still poorly understood, in this study we evaluated some parameters of oxidative stress, namely thiobarbituric acid-reactive substances (TBARS), total antioxidant reactivity (TAR) and total antioxidant status (TAS) in plasma from treated MSUD patients presenting high and low plasma leucine levels. We verified a significant increase of TBARS (lipid peroxidation) and a decrease of TAR (capacity to rapidly react with free radicals) in plasma from treated MSUD patients with low and with high plasma levels of leucine compared to the control group. It was also verified that TAS (quantity of tissue antioxidants) was not altered in plasma from treated MSUD patients with low and high blood leucine levels. Finally, we found no correlation between leucine, valine and isoleucine levels with the various parameters of oxidative stress. These results are indicative that increased lipid oxidative damage and decreased antioxidant defenses occur in plasma of MSUD patients and that the accumulating branched-chain amino acids are probably not directly associated to oxidative stress in this disorder.

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Acknowledgements

This work was supported by grants from Brazilian National Research Council (CNPq), CAPES, FAPERGS, and FIPE/HCPA—Brazil.

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Authors and Affiliations

  1. Serviço de Genética Médica, HCPA, Rua Ramiro Barcelos, 2350, CEP 90.035-903, Porto Alegre, Rio Grande do Sul, Brazil

    Alethéa G. Barschak, Angela Sitta, Marion Deon, Moacir Wajner & Carmen R. Vargas

  2. Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil

    Alethéa G. Barschak, Angela Sitta, Marion Deon, Carlos S. Dutra-Filho, Moacir Wajner & Carmen R. Vargas

  3. Departamento de Análises, Faculdade de Farmácia, Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil

    Amanda T. Barden & Carmen R. Vargas

Authors
  1. Alethéa G. Barschak
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  2. Angela Sitta
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  3. Marion Deon
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  4. Amanda T. Barden
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  5. Carlos S. Dutra-Filho
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  6. Moacir Wajner
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  7. Carmen R. Vargas
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Corresponding author

Correspondence to Carmen R. Vargas.

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Barschak, A.G., Sitta, A., Deon, M. et al. Oxidative stress in plasma from maple syrup urine disease patients during treatment. Metab Brain Dis 23, 71–80 (2008). https://doi.org/10.1007/s11011-007-9077-y

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  • DOI: https://doi.org/10.1007/s11011-007-9077-y

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