Tags

Type your tag names separated by a space and hit enter

Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease.
Int J Dev Neurosci. 2003 Oct; 21(6):327-32.IJ

Abstract

Maple syrup urine disease (MSUD) is an inherited disorder caused by deficiency of branched-chain L-2-keto acid dehydrogenase complex activity. Affected patients present severe brain dysfunction manifested as convulsions, coma, psychomotor delay and mental retardation. However, the underlying mechanisms of these neurological findings are virtually unknown. In this study, we tested the in vitro effect of L-leucine, L-isoleucine and L-valine, the amino acids accumulating in MSUD, on the lipid peroxidation parameters chemiluminescence and thiobarbituric acid-reactive substances (TBA-RS), as well as on total radical-trapping antioxidant potential (TRAP) and total antioxidant reactivity (TAR) in cerebral cortex from 30-day-old rats. L-Leucine significantly increased chemiluminescence and TBA-RS measurements and markedly decreased TRAP and TAR values. L-Isoleucine increased chemiluminescence and decreased TRAP measurements, but TAR and TBA-RS levels were not altered by the amino acid. Finally, TRAP measurement was diminished by L-valine. The results indicate a stimulation of lipid peroxidation and a reduction of brain capacity to efficiently modulate the damage associated with an increased production of free radicals by the branched-chain amino acids (BCAAs) accumulated in MSUD. It is therefore tempting to speculate that oxidative stress may be implicated in the brain damage found in MSUD patients.

Authors+Show Affiliations

Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600 Anexo, CEP 90035-003 Porto Alegre, RS, 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

12927581

Citation

Bridi, Raquel, et al. "Induction of Oxidative Stress in Rat Brain By the Metabolites Accumulating in Maple Syrup Urine Disease." International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience, vol. 21, no. 6, 2003, pp. 327-32.
Bridi R, Araldi J, Sgarbi MB, et al. Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease. Int J Dev Neurosci. 2003;21(6):327-32.
Bridi, R., Araldi, J., Sgarbi, M. B., Testa, C. G., Durigon, K., Wajner, M., & Dutra-Filho, C. S. (2003). Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease. International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience, 21(6), 327-32.
Bridi R, et al. Induction of Oxidative Stress in Rat Brain By the Metabolites Accumulating in Maple Syrup Urine Disease. Int J Dev Neurosci. 2003;21(6):327-32. PubMed PMID: 12927581.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease. AU - Bridi,Raquel, AU - Araldi,Janaína, AU - Sgarbi,Miriam B, AU - Testa,Carla G, AU - Durigon,Karina, AU - Wajner,Moacir, AU - Dutra-Filho,Carlos Severo, PY - 2003/8/21/pubmed PY - 2003/10/31/medline PY - 2003/8/21/entrez SP - 327 EP - 32 JF - International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience JO - Int J Dev Neurosci VL - 21 IS - 6 N2 - Maple syrup urine disease (MSUD) is an inherited disorder caused by deficiency of branched-chain L-2-keto acid dehydrogenase complex activity. Affected patients present severe brain dysfunction manifested as convulsions, coma, psychomotor delay and mental retardation. However, the underlying mechanisms of these neurological findings are virtually unknown. In this study, we tested the in vitro effect of L-leucine, L-isoleucine and L-valine, the amino acids accumulating in MSUD, on the lipid peroxidation parameters chemiluminescence and thiobarbituric acid-reactive substances (TBA-RS), as well as on total radical-trapping antioxidant potential (TRAP) and total antioxidant reactivity (TAR) in cerebral cortex from 30-day-old rats. L-Leucine significantly increased chemiluminescence and TBA-RS measurements and markedly decreased TRAP and TAR values. L-Isoleucine increased chemiluminescence and decreased TRAP measurements, but TAR and TBA-RS levels were not altered by the amino acid. Finally, TRAP measurement was diminished by L-valine. The results indicate a stimulation of lipid peroxidation and a reduction of brain capacity to efficiently modulate the damage associated with an increased production of free radicals by the branched-chain amino acids (BCAAs) accumulated in MSUD. It is therefore tempting to speculate that oxidative stress may be implicated in the brain damage found in MSUD patients. SN - 0736-5748 UR - https://www.unboundmedicine.com/medline/citation/12927581/Induction_of_oxidative_stress_in_rat_brain_by_the_metabolites_accumulating_in_maple_syrup_urine_disease_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0736574803000741 DB - PRIME DP - Unbound Medicine ER -