M Wajner

Summary

Affiliation: Porto Alegre
Country: Brazil

Publications

  1. ncbi request reprint The role of oxidative damage in the neuropathology of organic acidurias: insights from animal studies
    M 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
  2. doi request reprint Disruption of mitochondrial homeostasis in organic acidurias: insights from human and animal studies
    Moacir Wajner
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, UFRGS, Porto Alegre, RS, Brazil
    J Bioenerg Biomembr 43:31-8. 2011
  3. doi request reprint Selective screening for organic acidemias by urine organic acid GC-MS analysis in Brazil: fifteen-year experience
    Moacir Wajner
    Servico de Genetica Medica, Hospital de Clinicas, Porto Alegre, RS, Brazil
    Clin Chim Acta 400:77-81. 2009
  4. ncbi request reprint Detection of organic acidemias in Brazil
    Moacir Wajner
    Servico de Genetica Medica, Hospital de Clinicas, Porto Alegre, RS, 90 035 003, Brazil
    Arch Med Res 33:581-5. 2002
  5. ncbi request reprint A Bayesian neural network approach for modelling censored data with an application to prognosis after surgery for breast cancer
    P 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
  6. ncbi request reprint Na+, K+ ATPase activity is markedly reduced by cis-4-decenoic acid in synaptic plasma membranes from cerebral cortex of rats
    D R de Assis
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, UFRGS, Porto Alegre, RS, Brazil
    Exp Neurol 197:143-9. 2006
  7. ncbi request reprint Inhibition of mitochondrial creatine kinase activity from rat cerebral cortex by methylmalonic acid
    P 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
  8. ncbi request reprint 3-Hydroxyglutaric acid moderately impairs energy metabolism in brain of young rats
    A Latini
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade Federal de Rio Grande do Sul, Anexo, CEP 90035 003, Porto Alegre, RS, Brazil
    Neuroscience 135:111-20. 2005
  9. ncbi request reprint Investigation of oxidative stress parameters in treated phenylketonuric patients
    A Sitta
    Servico de Genetica Medica, HCPA, Rua Ramiro Barcelos, 2350 CEP, 90 035 903, Porto Alegre, RS, Brasil
    Metab Brain Dis 21:287-96. 2006
  10. ncbi request reprint Evidence for oxidative stress in tissues derived from succinate semialdehyde dehydrogenase-deficient mice
    A Latini
    Departamento de Bioquimica, Centro de Ciencias Biologicas, Universidade Federal de Santa Catarina, Florianopolis, SC, Brazil
    J Inherit Metab Dis 30:800-10. 2007

Collaborators

Detail Information

Publications46

  1. ncbi request reprint The role of oxidative damage in the neuropathology of organic acidurias: insights from animal studies
    M 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
    ....
  2. doi request reprint Disruption of mitochondrial homeostasis in organic acidurias: insights from human and animal studies
    Moacir 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...
  3. doi request reprint Selective screening for organic acidemias by urine organic acid GC-MS analysis in Brazil: fifteen-year experience
    Moacir Wajner
    Servico de Genetica Medica, Hospital de Clinicas, Porto Alegre, RS, Brazil
    Clin Chim Acta 400:77-81. 2009
    ....
  4. ncbi request reprint Detection of organic acidemias in Brazil
    Moacir 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...
  5. ncbi request reprint A Bayesian neural network approach for modelling censored data with an application to prognosis after surgery for breast cancer
    P 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...
  6. ncbi request reprint Na+, K+ ATPase activity is markedly reduced by cis-4-decenoic acid in synaptic plasma membranes from cerebral cortex of rats
    D R de Assis
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, UFRGS, Porto Alegre, RS, Brazil
    Exp Neurol 197:143-9. 2006
    ....
  7. ncbi request reprint Inhibition of mitochondrial creatine kinase activity from rat cerebral cortex by methylmalonic acid
    P 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
    ....
  8. ncbi request reprint 3-Hydroxyglutaric acid moderately impairs energy metabolism in brain of young rats
    A Latini
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade Federal de Rio Grande do Sul, Anexo, CEP 90035 003, Porto Alegre, RS, Brazil
    Neuroscience 135:111-20. 2005
    ....
  9. ncbi request reprint Investigation of oxidative stress parameters in treated phenylketonuric patients
    A Sitta
    Servico de Genetica Medica, 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...
  10. ncbi request reprint Evidence for oxidative stress in tissues derived from succinate semialdehyde dehydrogenase-deficient mice
    A 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...
  11. ncbi request reprint Inhibition of in vitro CO2 production and lipid synthesis by 2-hydroxybutyric acid in rat brain
    A 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...
  12. ncbi request reprint Inhibition of energy metabolism by 2-methylacetoacetate and 2-methyl-3-hydroxybutyrate in cerebral cortex of developing rats
    R B Rosa
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, 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...
  13. ncbi request reprint Effects of histidine and imidazolelactic acid on various parameters of the oxidative stress in cerebral cortex of young rats
    C M Tansini
    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
    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...
  14. ncbi request reprint In vitro stimulation of oxidative stress in cerebral cortex of rats by the guanidino compounds accumulating in hyperargininemia
    A 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...
  15. ncbi request reprint Inhibition of glutamate uptake into synaptic vesicles from rat brain by 3-nitropropionic acid in vitro
    R 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...
  16. ncbi request reprint Effects of methylmalonic and propionic acids on glutamate uptake by synaptosomes and synaptic vesicles and on glutamate release by synaptosomes from cerebral cortex of rats
    A 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
    ....
  17. ncbi request reprint Chronic early leucine administration induces behavioral deficits in rats
    C 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...
  18. ncbi request reprint Nitric oxide synthase inhibition by L-NAME prevents the decrease of Na+,K+-ATPase activity in midbrain of rats subjected to arginine administration
    A 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...
  19. ncbi request reprint Promotion of oxidative stress by 3-hydroxyglutaric acid in rat striatum
    A Latini
    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, 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...
  20. ncbi request reprint Inhibition of Na+,K+-ATPase activity from rat hippocampus by proline
    Z L Pontes
    , ICBS, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
    Neurochem Res 26:1321-6. 2001
    ....
  21. ncbi request reprint Pharmacological evidence that alpha-ketoisovaleric acid induces convulsions through GABAergic and glutamatergic mechanisms in rats
    A 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...
  22. ncbi request reprint Oxidative stress in patients with phenylketonuria
    L 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...
  23. ncbi request reprint Creatine prevents behavioral alterations caused by methylmalonic acid administration into the hippocampus of rats in the open field task
    V Vasques
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
    J Neurol Sci 244:23-9. 2006
    ....
  24. ncbi request reprint Cytoskeleton as a potential target in the neuropathology of maple syrup urine disease: insight from animal studies
    R 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
    ....
  25. ncbi request reprint L-pyroglutamic acid inhibits energy production and lipid synthesis in cerebral cortex of young rats in vitro
    A 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...
  26. ncbi request reprint Effect of propionic and methylmalonic acids on the high molecular weight neurofilament subunit (NF-H) in rat cerebral cortex
    L Vivian
    Universidade Federal do Rio Grande do Sul, Instituto de Ciencias Basicas da Saude, Departamento de Bioquimica, 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...
  27. ncbi request reprint Reduction of large neutral amino acid levels in plasma and brain of hyperleucinemic rats
    P 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
    ....
  28. ncbi request reprint D-2-hydroxyglutaric acid inhibits creatine kinase activity from cardiac and skeletal muscle of young rats
    C 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
    ..However, the underlying mechanisms of muscle injury in this disorder are virtually unknown...
  29. ncbi request reprint Reduction of large neutral amino acid concentrations in plasma and CSF of patients with maple syrup urine disease during crises
    M Wajner
    Servico de Genetica Medica, Hospital de Clinicas de Porto Alegre, Porto Alegre RS, Brazil
    J Inherit Metab Dis 23:505-12. 2000
    ....
  30. ncbi request reprint The influence of organic acids on the proliferation of human peripheral lymphocytes activated by concanavalin A and pokeweed mitogen
    K 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...
  31. doi request reprint Oxidative stress in phenylketonuric patients
    C 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...
  32. ncbi request reprint 3-hydroxyglutaric acid enhances glutamate uptake into astrocytes from cerebral cortex of young rats
    M E S Frizzo
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, UFRGS, Porto Alegre, RS, CEP 900035 003, Brazil
    Neurochem Int 44:345-53. 2004
    ....
  33. ncbi request reprint Effect of hyperprolinemia on acetylcholinesterase and butyrylcholinesterase activities in rat
    D Delwing
    Departamento de Bioquimica, ICBS, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
    Amino Acids 28:305-8. 2005
    ....
  34. doi request reprint Evidence that L-carnitine and selenium supplementation reduces oxidative stress in phenylketonuric patients
    A Sitta
    Servico de Genetica Medica, HCPA, UFRGS, Rua Ramiro Barcelos, Porto Alegre, RS, Brazil
    Cell Mol Neurobiol 31:429-36. 2011
    ....
  35. ncbi request reprint Reduced Na(+), K(+)-ATPase activity in erythrocyte membranes from patients with phenylketonuria
    M Bedin
    , , Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
    Pediatr Res 50:56-60. 2001
    ....
  36. ncbi request reprint Detection of inborn errors of metabolism in unselected patients from pediatric intensive care units in Porto Alegre, Brazil: evaluation of screening techniques
    C M Wannmacher
    , Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brasil
    Braz J Med Biol Res 20:11-23. 1987
    ....
  37. ncbi request reprint Inhibition of rat brain Na+, K+-ATPase activity induced by homocysteine is probably mediated by oxidative stress
    E 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...
  38. ncbi request reprint Evidence that oxidative stress is increased in patients with X-linked adrenoleukodystrophy
    C 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...
  39. ncbi request reprint Modulation of glutamatergic and GABAergic neurotransmission in glutaryl-CoA dehydrogenase deficiency
    M 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...
  40. ncbi request reprint Inhibition of the mitochondrial respiratory chain complex activities in rat cerebral cortex by methylmalonic acid
    A M Brusque
    Departamento de Bioquimica, Instituto de Ciencias Basicas da Saude, UFRGS, Rua Ramiro Barcelos 2600, CEP 90 035 003 Porto Alegre, RS, Brazil
    Neurochem Int 40:593-601. 2002
    ....
  41. ncbi request reprint Creatine protects against the convulsive behavior and lactate production elicited by the intrastriatal injection of methylmalonate
    L F F Royes
    Department of Chemistry, Federal University of Santa Maria, 97105 900 Santa Maria, RS, Brazil
    Neuroscience 118:1079-90. 2003
    ....
  42. ncbi request reprint 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 cortex
    R 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...
  43. ncbi request reprint Intrastriatal administration of 3-hydroxyglutaric acid induces convulsions and striatal lesions in rats
    C 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...
  44. ncbi request reprint Isolated 3-methylcrotonyl-coenzyme A carboxylase deficiency in a child with metabolic stroke
    L Pinto
    Serviços de Genética Clínica e Neurologia, 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...
  45. ncbi request reprint Animal models for glutaryl-CoA dehydrogenase deficiency
    D 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...
  46. doi request reprint Methylmalonate inhibits succinate-supported oxygen consumption by interfering with mitochondrial succinate uptake
    S 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
    ....