Research Topics
Species | M WajnerSummaryAffiliation: Porto Alegre Country: Brazil Publications
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Detail Information
Publications
The role of oxidative damage in the neuropathology of organic acidurias: insights from animal studiesM Wajner
Servico de Genetica Medica, Hospital de Clinicas de Porto Alegre, Rua Ramiro Barcelos 2350, 90035 003 Porto Alegre, RS, Brazil
J Inherit Metab Dis 27:427-48. 2004....
Disruption of mitochondrial homeostasis in organic acidurias: insights from human and animal studiesMoacir Wajner
Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, UFRGS, Porto Alegre, RS, Brazil
J Bioenerg Biomembr 43:31-8. 2011..The elucidation of the mechanisms of toxicity of these acidic compounds offers new perspectives for potential novel adjuvant therapeutic strategies in selected disorders of this group...- Detection of organic acidemias in BrazilMoacir Wajner
Servico de Genetica Medica, Hospital de Clinicas, Porto Alegre, RS, 90 035 003, Brazil
Arch Med Res 33:581-5. 2002..They are considered the most frequent metabolic disorders among severely ill children. Patients frequently present acute symptoms in early life. Metabolic acidosis and neurologic symptoms are the most common signs... - Selective screening for organic acidemias by urine organic acid GC-MS analysis in Brazil: fifteen-year experienceMoacir Wajner
Servico de Genetica Medica, Hospital de Clinicas, Porto Alegre, RS, Brazil
Clin Chim Acta 400:77-81. 2009.... - A Bayesian neural network approach for modelling censored data with an application to prognosis after surgery for breast cancerP J G Lisboa
School of Computing and Mathematical Sciences, Liverpool John Moores University, Byrom Street, Liverpool L3 3AF, UK
Artif Intell Med 28:1-25. 2003..With automatic model selection, the regularised neural network is more conservative than the default stepwise forward selection procedure implemented by SPSS with the Akaike Information Criterion... - 3-Hydroxyglutaric acid moderately impairs energy metabolism in brain of young ratsA Latini
, , Universidade Federal de Rio Grande do Sul, Anexo, CEP 90035-003, Porto Alegre, RS, Brazil
Neuroscience 135:111-20. 2005.... - Inhibition of energy metabolism by 2-methylacetoacetate and 2-methyl-3-hydroxybutyrate in cerebral cortex of developing ratsR B Rosa
, , Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
J Inherit Metab Dis 28:501-15. 2005..If the in vitro effects detected in the present study also occur in vivo, it is tempting to speculate that they may contribute, at least in part, to the neurological dysfunction found in these disorders... - Investigation of oxidative stress parameters in treated phenylketonuric patientsA Sitta
, HCPA, Rua Ramiro Barcelos, 2350 CEP, 90.035-903, Porto Alegre, RS, Brasil
Metab Brain Dis 21:287-96. 2006..In contrast, CAT and SOD activities were not altered in PKU patients. These results show that oxidative stress occurs in PKU patients and that this pathogenic process is probably not directly correlated to Phe blood levels... - Inhibition of mitochondrial creatine kinase activity from rat cerebral cortex by methylmalonic acidP F Schuck
Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600 Anexo, CEP 90035 003, Porto Alegre, RS, Brazil
Neurochem Int 45:661-7. 2004.... - Evidence for oxidative stress in tissues derived from succinate semialdehyde dehydrogenase-deficient miceA Latini
Departamento de Bioquimica, Centro de Ciencias Biologicas, Universidade Federal de Santa Catarina, Florianopolis, SC, Brazil
J Inherit Metab Dis 30:800-10. 2007..Our data showing an imbalance between tissue antioxidant defences and oxidative attack strongly indicate that oxidative stress is involved in the pathophysiology of SSADH deficiency in mice, and likely the corresponding human disorder... - Inhibition of in vitro CO2 production and lipid synthesis by 2-hydroxybutyric acid in rat brainA R Silva
, , Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brasil
Braz J Med Biol Res 34:627-31. 2001..It is possible that these findings may be associated with the neuropathophysiology of the situations where 2-hydroxybutyric acid is accumulated... - Na+, K+ ATPase activity is markedly reduced by cis-4-decenoic acid in synaptic plasma membranes from cerebral cortex of ratsD R de Assis
, , UFRGS, Porto Alegre, RS, Brazil
Exp Neurol 197:143-9. 2006.... - Inhibition of glutamate uptake into synaptic vesicles from rat brain by 3-nitropropionic acid in vitroR G Tavares
, , Universidade Federal do Rio Grande do Sul, 90035-003 Porto Alegre, RS, Brazil
Exp Neurol 172:250-4. 2001..These findings indicate that low concentrations of 3-NP are able to selectively prevent vesicular glutamate storage, and this may represent at least one of the mechanisms responsible for the neurotoxic effects of 3-NP... - In vitro stimulation of oxidative stress in cerebral cortex of rats by the guanidino compounds accumulating in hyperargininemiaA T Wyse
Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600 Anexo, CEP 90035 003, Porto Alegre RS, Brazil
Brain Res 923:50-7. 2001..The results suggest that oxidative stress caused by guanidino compounds may be involved in the brain dysfunction amongst other potential pathophysiological mechanisms observed in hyperargininemia... - Effects of histidine and imidazolelactic acid on various parameters of the oxidative stress in cerebral cortex of young ratsC M Tansini
, , Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600 Anexo, 90035-003 Porto Alegre, RS, Brazil
Int J Dev Neurosci 22:67-72. 2004..5-1 mM). These results suggest that in vitro oxidative stress is elicited by histidine but only at supraphysiological concentrations... - Effects of methylmalonic and propionic acids on glutamate uptake by synaptosomes and synaptic vesicles and on glutamate release by synaptosomes from cerebral cortex of ratsA M Brusque
, , Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos 2600, Anexo CEP 90.035-003, RS, Porto Alegre, Brazil
Brain Res 920:194-201. 2001.... - Chronic early leucine administration induces behavioral deficits in ratsC F Mello
Departamento de Quimica, CCNE, Universidade Federal de Santa Maria, RS, Brazil
Life Sci 65:747-55. 1999..Chronic early leucine treatment impaired acquisition of a two-way shuttle avoidance task and altered habituation to an open field. Our results suggest that early postnatal leucine administration induces long-lasting behavioral deficits... - Nitric oxide synthase inhibition by L-NAME prevents the decrease of Na+,K+-ATPase activity in midbrain of rats subjected to arginine administrationA T Wyse
Departamento de Bioquimica, ICBS, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil
Neurochem Res 26:515-20. 2001..These results demonstrate that in vivo arginine administration reduces Na(+),K(+)-ATPase activity possibly through free radical generation induced by NO formation... - Promotion of oxidative stress by 3-hydroxyglutaric acid in rat striatumA Latini
, , Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600 - Anexo, CEP 90035-003, Porto Alegre, RS, Brasil
J Inherit Metab Dis 28:57-67. 2005..Furthermore, the results also reflect an increase of lipid peroxidation, probably secondary to 3-OH-GA-induced free radical production. Thus, it may be presumed that oxidative stress is involved in the neuropathology in GDD... - Inhibition of Na+,K+-ATPase activity from rat hippocampus by prolineZ L Pontes
, ICBS, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
Neurochem Res 26:1321-6. 2001.... - Pharmacological evidence that alpha-ketoisovaleric acid induces convulsions through GABAergic and glutamatergic mechanisms in ratsA S Coitinho
Universidade Federal do Rio Grande do Sul, , , Rua Ramiro Barcelos 2600, RS 90035-003, Porto Alegre, Brazil
Brain Res 894:68-73. 2001..The authors suggest that KIV may play an important role in the convulsions observed in MSUD, and highlight its relevance to the understanding of the pathophysiology of the neurological dysfunction of MSUD patients... - Oxidative stress in patients with phenylketonuriaL R Sirtori
Medical Genetics Service, HCPA, Rua Ramiro Barcelos, 2350 CEP 90.035-003, Porto Alegre, RS, Brazil
Biochim Biophys Acta 1740:68-73. 2005..The results also showed a decrease of erythrocyte GSH-Px activity. Therefore, it is presumed that oxidative stress is involved in the pathophysiology of the tissue damage found in PKU... - Creatine prevents behavioral alterations caused by methylmalonic acid administration into the hippocampus of rats in the open field taskV Vasques
, , Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
J Neurol Sci 244:23-9. 2006.... - Cytoskeleton as a potential target in the neuropathology of maple syrup urine disease: insight from animal studiesR Pessoa Pureur
Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos 2600 anexo, 90035 003, Porto Alegre, RS, Brazil
J Inherit Metab Dis 30:664-72. 2007.... - L-pyroglutamic acid inhibits energy production and lipid synthesis in cerebral cortex of young rats in vitroA R Silva
, , Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
Neurochem Res 26:1277-83. 2001..The results strongly indicate that PGA impairs brain energy production. If these effects also occur in humans, it is possible that they may contribute to the neuropathology of patients affected by these diseases... - Effect of propionic and methylmalonic acids on the high molecular weight neurofilament subunit (NF-H) in rat cerebral cortexL Vivian
Universidade Federal do Rio Grande do Sul, , , 90035-003 Porto Alegre, RS, Brazil
Neurochem Res 27:1691-7. 2002..Furthermore, treatments significantly increased the total amount of NF-H in 12-day-old rats. These findings indicate that PA and MMA alter the dynamic regulation of NF-H assembly in the cytoskeletal fraction... - Reduction of large neutral amino acid levels in plasma and brain of hyperleucinemic ratsP Araujo
Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos 2600, CEP 90 035 003, Porto Alegre, RS, Brazil
Neurochem Int 38:529-37. 2001.... - D-2-hydroxyglutaric acid inhibits creatine kinase activity from cardiac and skeletal muscle of young ratsC G da Silva
Department of Biochemistry, Institute of Basic Sciences and Health, UFRGS, Porto Alegre, RS, Brazil
Eur J Clin Invest 33:840-7. 2003.... - Reduction of large neutral amino acid concentrations in plasma and CSF of patients with maple syrup urine disease during crisesM Wajner
Servico de Genetica Medica, Hospital de Clinicas de Porto Alegre, Porto Alegre RS, Brazil
J Inherit Metab Dis 23:505-12. 2000.... - The influence of organic acids on the proliferation of human peripheral lymphocytes activated by concanavalin A and pokeweed mitogenK D Santos
Departamento de Bioquimica, UFRGS, Unidade de Genética Médica, HCPA, RS Porto Alegre, Brazil
Int J Immunopharmacol 18:761-9. 1996..The present study therefore demonstrated that some organic acids modulate DNA synthesis in Con A- and PWM-stimulated human lymphocytes... - Oxidative stress in phenylketonuric patientsC R Vargas
Programa de Pós Graduação em Ciências Biológicas Bioquímica, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
Mol Genet Metab 104:S97-9. 2011..Therefore, antioxidants could be considered an adjuvant therapy in phenylketonuria... - 3-hydroxyglutaric acid enhances glutamate uptake into astrocytes from cerebral cortex of young ratsM E S Frizzo
, , UFRGS, Porto Alegre, RS, CEP 900035-003, Brazil
Neurochem Int 44:345-53. 2004.... - Effect of hyperprolinemia on acetylcholinesterase and butyrylcholinesterase activities in ratD Delwing
, ICBS, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
Amino Acids 28:305-8. 2005.... - Evidence that L-carnitine and selenium supplementation reduces oxidative stress in phenylketonuric patientsA Sitta
Servico de Genetica Medica, HCPA, UFRGS, Rua Ramiro Barcelos, Porto Alegre, RS, Brazil
Cell Mol Neurobiol 31:429-36. 2011.... - Reduced Na(+), K(+)-ATPase activity in erythrocyte membranes from patients with phenylketonuriaM Bedin
, , Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
Pediatr Res 50:56-60. 2001.... - Detection of inborn errors of metabolism in unselected patients from pediatric intensive care units in Porto Alegre, Brazil: evaluation of screening techniquesC M Wannmacher
, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brasil
Braz J Med Biol Res 20:11-23. 1987.... - Inhibition of rat brain Na+, K+-ATPase activity induced by homocysteine is probably mediated by oxidative stressE L Streck
, ICBS, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil
Neurochem Res 26:1195-200. 2001..These results suggest that inhibition of this enzyme activity by homocysteine is possibly mediated by free radicals and may contribute to the neurological dysfunction found in homocystinuric patients... - Evidence that oxidative stress is increased in patients with X-linked adrenoleukodystrophyC R Vargas
Department of Clinical Analysis, Pharmacy Faculty, UFRGS, Rua Ramiro Barcelos, 2350 CEP 90 035 003, Porto Alegre, RS, Brazil
Biochim Biophys Acta 1688:26-32. 2004..We also observed a significant increase of erythrocytes GPx activity and of catalase and SOD activities in fibroblasts from the patients studied. It is therefore proposed that oxidative stress may be involved in pathophysiology of X-ALD... - Modulation of glutamatergic and GABAergic neurotransmission in glutaryl-CoA dehydrogenase deficiencyM Wajner
Department of Biochemistry, Instituto de Ciencias Basicas da Saude, Federal University of Rio Grande do Sul, Porto Alegre, RS, Brazil
J Inherit Metab Dis 27:825-8. 2004..In conclusion, glutaric acid and 3-hydroxyglutaric acid induce an imbalance in glutamatergic and GABAergic neurotransmission... - Inhibition of the mitochondrial respiratory chain complex activities in rat cerebral cortex by methylmalonic acidA M Brusque
, , UFRGS, Rua Ramiro Barcelos 2600, CEP 90.035-003 Porto Alegre, RS, Brazil
Neurochem Int 40:593-601. 2002.... - Effect of the branched-chain alpha-ketoacids accumulating in maple syrup urine disease on the high molecular weight neurofilament subunit (NF-H) in rat cerebral cortexR Pessoa-Pureur
Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brasil
Metab Brain Dis 17:65-75. 2002..Therefore we may suggest that cytoskeletal disorganization may be one of the factors associated with the neurodegeneration characteristic of MSUD disease... - Creatine protects against the convulsive behavior and lactate production elicited by the intrastriatal injection of methylmalonateL F F Royes
Department of Chemistry, Federal University of Santa Maria, 97105-900 Santa Maria, RS, Brazil
Neuroscience 118:1079-90. 2003.... - Intrastriatal administration of 3-hydroxyglutaric acid induces convulsions and striatal lesions in ratsC F de Mello
Department of Chemistry, University of Santa Maria, Santa Maria, Brazil
Brain Res 916:70-5. 2001..These results support the hypothesis of NMDA receptor-mediated excitotoxic cell damage in glutaryl-CoA dehydrogenase deficiency and represent the basis for the development of new neuroprotective treatment strategies... - Isolated 3-methylcrotonyl-coenzyme A carboxylase deficiency in a child with metabolic strokeL Pinto
, FFFCMPA, Brazil
J Inherit Metab Dis 29:205-6. 2006..We report a 3-year-old boy with isolated 3-methylcrotonyl-coenzyme A deficiency with unexpectedly severe presentation, seizures and history of cerebral ischae-mic episode... - Animal models for glutaryl-CoA dehydrogenase deficiencyD M Koeller
Department of Pediatrics, Oregon Health and Science University, Portland, Oregon 97239, USA
J Inherit Metab Dis 27:813-8. 2004..Unravelling these mechanisms may be the key to understanding the pathophysiology of this unique disease and to the development of neuroprotective strategies... - Methylmalonate inhibits succinate-supported oxygen consumption by interfering with mitochondrial succinate uptakeS R Mirandola
Departamento de Patologia Clinica, Faculdade de Ciencias Medicas, Universidade Estadual de Campinas, Campinas, SP 13083 887, Brazil
J Inherit Metab Dis 31:44-54. 2008....