title: 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.
abstract: 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.
authors.affiliation: Departamento de Bioquímica, Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600 anexo, Porto Alegre, RS, 90035-003, Brazil.
authors.forename: Itiane Diehl
authors.initials: ID
authors.lastname: de Franceschi
authors.forename: Elenara
authors.initials: E
authors.lastname: Rieger
authors.forename: Alessandra Pinto
authors.initials: AP
authors.lastname: Vargas
authors.forename: Denise Bertin
authors.initials: DB
authors.lastname: Rojas
authors.forename: Aline Guimarães
authors.initials: AG
authors.lastname: Campos
authors.forename: Virginia Cielo
authors.initials: VC
authors.lastname: Rech
authors.forename: Luciane Rosa
authors.initials: LR
authors.lastname: Feksa
authors.forename: Clóvis Milton Duval
authors.initials: CM
authors.lastname: Wannmacher
mesh_terms: D000818:Animals; D000975:Antioxidants / Q000378:metabolism; D002540:Cerebral Cortex / Q000187:drug effects / Q000378:metabolism*; D003401:Creatine / Q000494:pharmacology; D005260:Female; D006624:Hippocampus / Q000187:drug effects / Q000378:metabolism*; D007774:Lactation / Q000187:drug effects; D007930:Leucine / Q000494:pharmacology*; D008297:Male; D008375:Maple Syrup Urine Disease / Q000503:physiopathology*; D018384:Oxidative Stress / Q000187:drug effects; D010770:Phosphotransferases / Q000378:metabolism*; D011247:Pregnancy; D011297:Prenatal Exposure Delayed Effects; D019289:Pyruvic Acid / Q000494:pharmacology; D051381:Rats; D017208:Rats, Wistar
chemical_list: D000975:Antioxidants; D019289:Pyruvic Acid; D010770:Phosphotransferases; D007930:Leucine; D003401:Creatine