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Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease.
Metab Brain Dis. 2006 Dec; 21(4):279-86.MB

Abstract

Maple syrup urine disease (MSUD) or branched-chain alpha-keto aciduria (BCKA) is an inherited disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKAD) activity. The blockage of this pathway leads to tissue accumulation of the branched-chain amino acids (BCAA) leucine, isoleucine and valine and their respective keto-acids. The clinical features presented by MSUD patients include ketoacidosis, convulsions, coma, psychomotor delay and mental retardation. The mechanism of brain damage in this disease is still poorly understood. However, an increase in lipid peroxidation in vitro in cerebral cortex of young rats as well as a decrease in the antioxidant defenses has been previously observed. In the present work we evaluated different oxidative stress parameters, named reactive species of thiobarbituric acid (TBARS), total antioxidant reactivity (TAR) and total antioxidant status (TAS) in plasma of MSUD patients in order to evaluate whether oxidative stress is involved in this disorder. We verified a marked increase of plasma TBARS measurements, which is indicative of increased lipid peroxidation, as well as a decrease on plasma TAR reflecting a deficient capacity to efficiently modulate the damage associated with an increased production of reactive species. In contrast, TAS was not changed indicating that the total content of antioxidants in plasma of patients affected by MSUD was not altered. These results suggest that free radical generation is elicited in MSUD and is possibly involved in the pathophysiology of the tissue damage found in this disorder.

Authors+Show Affiliations

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.No 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

17091400

Citation

Barschak, Alethéa G., et al. "Evidence That Oxidative Stress Is Increased in Plasma From Patients With Maple Syrup Urine Disease." Metabolic Brain Disease, vol. 21, no. 4, 2006, pp. 279-86.
Barschak AG, Sitta A, Deon M, et al. Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease. Metab Brain Dis. 2006;21(4):279-86.
Barschak, A. G., Sitta, A., Deon, M., de Oliveira, M. H., Haeser, A., Dutra-Filho, C. S., Wajner, M., & Vargas, C. R. (2006). Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease. Metabolic Brain Disease, 21(4), 279-86.
Barschak AG, et al. Evidence That Oxidative Stress Is Increased in Plasma From Patients With Maple Syrup Urine Disease. Metab Brain Dis. 2006;21(4):279-86. PubMed PMID: 17091400.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease. AU - Barschak,Alethéa G, AU - Sitta,Angela, AU - Deon,Marion, AU - de Oliveira,Marcella H, AU - Haeser,Alexsandro, AU - Dutra-Filho,Carlos S, AU - Wajner,Moacir, AU - Vargas,Carmen R, Y1 - 2006/11/08/ PY - 2006/01/17/received PY - 2006/05/08/accepted PY - 2006/11/9/pubmed PY - 2007/2/13/medline PY - 2006/11/9/entrez SP - 279 EP - 86 JF - Metabolic brain disease JO - Metab Brain Dis VL - 21 IS - 4 N2 - Maple syrup urine disease (MSUD) or branched-chain alpha-keto aciduria (BCKA) is an inherited disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKAD) activity. The blockage of this pathway leads to tissue accumulation of the branched-chain amino acids (BCAA) leucine, isoleucine and valine and their respective keto-acids. The clinical features presented by MSUD patients include ketoacidosis, convulsions, coma, psychomotor delay and mental retardation. The mechanism of brain damage in this disease is still poorly understood. However, an increase in lipid peroxidation in vitro in cerebral cortex of young rats as well as a decrease in the antioxidant defenses has been previously observed. In the present work we evaluated different oxidative stress parameters, named reactive species of thiobarbituric acid (TBARS), total antioxidant reactivity (TAR) and total antioxidant status (TAS) in plasma of MSUD patients in order to evaluate whether oxidative stress is involved in this disorder. We verified a marked increase of plasma TBARS measurements, which is indicative of increased lipid peroxidation, as well as a decrease on plasma TAR reflecting a deficient capacity to efficiently modulate the damage associated with an increased production of reactive species. In contrast, TAS was not changed indicating that the total content of antioxidants in plasma of patients affected by MSUD was not altered. These results suggest that free radical generation is elicited in MSUD and is possibly involved in the pathophysiology of the tissue damage found in this disorder. SN - 0885-7490 UR - https://www.unboundmedicine.com/medline/citation/17091400/Evidence_that_oxidative_stress_is_increased_in_plasma_from_patients_with_maple_syrup_urine_disease_ L2 - https://doi.org/10.1007/s11011-006-9030-5 DB - PRIME DP - Unbound Medicine ER -