Neurochemical Research

, Volume 38, Issue 3, pp 632–643 | Cite as

Effect of Leucine Administration to Female Rats During Pregnancy and Lactation on Oxidative Stress and Enzymes Activities of Phosphoryltransfer Network in Cerebral Cortex and Hippocampus of the Offspring

  • Itiane Diehl de Franceschi
  • Elenara Rieger
  • Alessandra Pinto Vargas
  • Denise Bertin Rojas
  • Aline Guimarães Campos
  • Virginia Cielo Rech
  • Luciane Rosa Feksa
  • Clóvis Milton Duval Wannmacher
Original Paper


Maple Syrup Urine Disease is an inborn error of metabolism caused by severe deficiency in the activity of branched-chain α-keto acid dehydrogenase complex. Neurological disorder is common in patients with maple syrup urine disease. Although leucine is considered the main toxic metabolite, the mechanisms underlying the neuropathology of brain injury are poorly understood. In the present study, we evaluated the possible preventive effect of the co-administration of creatine plus pyruvate on the effects elicited by leucine administration to female Wistar rats during pregnancy and lactation on some oxidative stress parameters as well as the activities of some enzymes involved in the phosphoryltransfer network in the brain cortex and hippocampus of the offspring at 21 days of age. Leucine administration induced oxidative stress and altered the activities of pyruvate kinase, adenylate kinase, mitochondrial and cytosolic creatine kinase. Co-administration of creatine plus pyruvate was partially effective in the prevention of some alterations provoked by leucine administration on the oxidative stress but not in the enzymes of phosphoryltransfer network. These results suggest that non-treated maternal hyperleucinemia may be toxic to the brain of the offspring.


l-Leucine Maternal hyperleucinemia Oxidative stress Phosphoryltransfer network Creatine Pyruvate 



This work was supported by the research grants from Programa de Núcleos de Excelência (PRONEX), Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES), Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq), Fundação de Amparo à Pesquisa do Estado do Rio Grande do Sul (FAPERGS) and FINEP Rede Instituto Brasileiro de Neurociência.


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Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Itiane Diehl de Franceschi
    • 1
  • Elenara Rieger
    • 1
  • Alessandra Pinto Vargas
    • 1
  • Denise Bertin Rojas
    • 1
  • Aline Guimarães Campos
    • 1
  • Virginia Cielo Rech
    • 2
  • Luciane Rosa Feksa
    • 3
  • Clóvis Milton Duval Wannmacher
    • 1
  1. 1.Departamento de Bioquímica, Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da SaúdeUniversidade Federal do Rio Grande do SulPorto AlegreBrazil
  2. 2.Laboratório de NanociênciasCentro Universitário Franciscano, UNIFRASanta MariaBrazil
  3. 3.Instituto de Ciências da Saúde-ICSUniversidade FeevaleNovo HamburgoBrazil

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