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Oxidative stress in plasma from maple syrup urine disease patients during treatment.
Metab Brain Dis. 2008 Mar; 23(1):71-80.MB

Abstract

Maple Syrup Urine Disease (MSUD) is an autosomal recessive metabolic disorder caused by a deficiency of branched-chain alpha-keto acid dehydrogenase complex activity leading to accumulation of the branched-chain amino acids leucine, isoleucine and valine and their corresponding branched-chain alpha-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.

Authors+Show Affiliations

Serviço de Genética Médica, HCPA, Rua Ramiro Barcelos 2350, Porto Alegre, Rio Grande do Sul, Brazil.No 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

18026828

Citation

Barschak, Alethéa G., et al. "Oxidative Stress in Plasma From Maple Syrup Urine Disease Patients During Treatment." Metabolic Brain Disease, vol. 23, no. 1, 2008, pp. 71-80.
Barschak AG, Sitta A, Deon M, et al. Oxidative stress in plasma from maple syrup urine disease patients during treatment. Metab Brain Dis. 2008;23(1):71-80.
Barschak, A. G., Sitta, A., Deon, M., Barden, A. T., Dutra-Filho, C. S., Wajner, M., & Vargas, C. R. (2008). Oxidative stress in plasma from maple syrup urine disease patients during treatment. Metabolic Brain Disease, 23(1), 71-80.
Barschak AG, et al. Oxidative Stress in Plasma From Maple Syrup Urine Disease Patients During Treatment. Metab Brain Dis. 2008;23(1):71-80. PubMed PMID: 18026828.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Oxidative stress in plasma from maple syrup urine disease patients during treatment. AU - Barschak,Alethéa G, AU - Sitta,Angela, AU - Deon,Marion, AU - Barden,Amanda T, AU - Dutra-Filho,Carlos S, AU - Wajner,Moacir, AU - Vargas,Carmen R, Y1 - 2007/11/17/ PY - 2006/12/06/received PY - 2007/08/22/accepted PY - 2007/11/21/pubmed PY - 2008/5/14/medline PY - 2007/11/21/entrez SP - 71 EP - 80 JF - Metabolic brain disease JO - Metab Brain Dis VL - 23 IS - 1 N2 - Maple Syrup Urine Disease (MSUD) is an autosomal recessive metabolic disorder caused by a deficiency of branched-chain alpha-keto acid dehydrogenase complex activity leading to accumulation of the branched-chain amino acids leucine, isoleucine and valine and their corresponding branched-chain alpha-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. SN - 0885-7490 UR - https://www.unboundmedicine.com/medline/citation/18026828/Oxidative_stress_in_plasma_from_maple_syrup_urine_disease_patients_during_treatment_ L2 - https://doi.org/10.1007/s11011-007-9077-y DB - PRIME DP - Unbound Medicine ER -