[000985058]
Deon, Marion et al. Urinary biomarkers of oxidative stress and plasmatic inflammatory profile in
phenylketonuric treated patients . In: International journal of developmental neuroscience, Oxford Vol. 47 part B (Dec. 2015), p. 259-265 , il.
[000965104]
Guerreiro, Gilian Batista Balbueno et al. Urinary biomarkers of oxidative damage in maple syrup urine disease : the L-carnitine role. In: International journal of developmental neuroscience, Oxford Vol. 42 (May 2015), p. 10-14 , il.
[000973458]
Mello, Mariana dos Santos et al. Increased oxidative stress in patients with 3-hydroxy-3-methylglutaric
aciduria . In: Molecular and cellular biochemistry, The Hague Vol. 402, no. 1 (Apr. 2015), p. 149-155 , digital. [000976823]
Mescka, Caroline Paula et al. Investigation of inflammatory profile in MSUD patients : benefit of L-carnitine supplementation. In: Metabolic brain disease, Amsterdam Vol. 30, no. 5 (Oct. 2015), p. 1167-1174 , digital. [000968599]
Mescka, Caroline Paula et al. L-Carnitine supplementation decreases DNA damage in treated MSUD patients . In: Mutation research. Fundamental and molecular mechanisms of mutagenesis, Amsterdam Vol. 775 ( May 2015), p. 43-47 , il.
[000973441]
Rosa, Mateus Struecker da et al. In vivo intracerebral administration of L-2-hydroxyglutaric acid provokes oxidative stress and histopathological alterations in striatum and cerebellum of adolescent rats . In: Free radical biology and medicine, Tarrytown, NY Vol. 83 (Jun. 2015), p. 201-213 , digital.
[000971764]
Vanzin, Camila Simioni et al. Lipid, oxidative and inflammatory profile and alterations in the enzymes paraoxonase and butyrylcholinesterase in plasma of patients with homocystinuria due CBS deficiency : the Vitamin B12 and folic acid importance. In: Cellular and molecular neurobiology, New York Vol. 35, No. 6 (Ago. 2015), p. 899-911 , il.
[000973460]
Zanatta, Angela et al. Ornithine in vivo administration disrupts redox homeostasis and decreases synaptic Na(+), K (+)-ATPase activity in cerebellum of adolescent rats : implications for the pathogenesis of hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome. In: Cellular and molecular neurobiology, New York Vol. 35, no. 6 (Aug. 2015), p. 797-806 , digital.
[000942130]
Busanello, Estela Natacha Brandt et al. Pristanic acid provokes lipid, protein, and dna oxidative damage and reduces the antioxidant defenses in cerebellum of young rats . In: Cerebellum, New York Vol. 13, n. 6 (Dec. 2014), p. 751–759.
[000942134]
Derks, Terry G.J. et al. Experimental evidence for protein oxidative damage and altered antioxidan defense in patients with medium-chain acyl-coa dehydrogenase deficiency . In: Journal of inherited metabolic disease, Lancaster Vol. 37, n. 5 (Sep. 2014), p. 783-789 , il.
[000934661]
Grings, Mateus et al. Sulfite disrupts brain mitochondrial energy homeostasis and induces mitochondrial permeability transition pore opening via thiol group modification . In: Biochimica et biophysica acta. Molecular basis of disease, Amsterdam : Elsevier Vol. 1842, no. 9 (Sept. 2014), p. 1413-1422 , il. [000933526]
Pierozan, Paula et al. Biochemical, histopathological and behavioral alterations caused by intrastriatal administration of quinolic acid to young rats . In: The FEBS journal, [Oxford, UK] Vol. 281, no. 8 (Apr. 2014), p. 2061-2073.
[000942141]
Ribas, Graziela de Oliveira Schmitt; Wajner, Moacir; Vargas, Carmen Regla. L-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders . In: Gene : international journal on genes and genomes. Amsterdam Vol. 533, n. 2 (Jan. 2014), p. 469-476 , il.
[000942137]
Sitta, Angela et al. Neurological damage in msud : the role of oxidative stress. In: Cellular and molecular neurobiology, New York Vol. 34, n. 2 (Mar. 2014), p. 157-165.
Vanzin, Camila Simioni et al. Homocysteine contribution to DNA damage in cystathionine
β-synthase-deficient patients . In: Gene : international journal on genes and genomes. Amsterdam Vol. 539, no. 2 (15 Apr. 2014), p. 270-274.
[000894666]
Busanello, Estela Natacha Brandt et al. Neurodevelopmental and cognitive behavior of glutaryl-CoA
dehydrogenase deficient knockout mice . In: Life sciences. [New York, NY] Vol. 92, Issue 2 (Feb. 2013), p. 137–142.
[000875120]
Busanello, Estela Natacha Brandt et al. Marked inhibition of Na+, K+ - ATPase activity and the respiratory chain by phytanic acid in cerebellum from young rats : possible underlying mechanisms of cerebellar ataxia in Refsum disease. In: Journal of bioenergetics and biomembranes, New York: Plenum Press, 2013 Vol. 45, issue 1-2 (Feb. 2013), p. 137-144.
[000881554]
Busanello, Estela Natacha Brandt et al. Disruption of mitochondrial homeostasis by phytanic acid in cerebellum of young rats . In: Cerebellum, London Vol. 12, no. 3 (Jun. 2013), p. 362-369.
[000909974]
Fernandes, Carolina Gonçalves et al. In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency induce oxidative stress in striatum of developing rats : a potential pathophysiological mechanism of striatal damage in this disorder. In: Molecular genetics and metabolism, Orlando Vol. 109, no. 2 (Jun. 2013), p. 144–153.
[000915474]
Grings, Mateus et al. Disturbance of brain energy and redox homeostasis provoked by sulfiteand thiosulfate : potential pathomechanisms involved in the neuropathology of sulfite oxidase deficiency. In: Gene :
international journal on genes and genomes. Amsterdam Vol. 531, no. 2 (Dec. 2013), p. 191-198. [000894669]
Mescka, Caroline Paula et al. Protein and lipid damage in maple syrup urine disease patients : l-carnitine effect. In: International journal of developmental neuroscience, [Oxford] Vol. 31, no. 1 (Feb. 2013), p. 21-24 , il.
Moura, Alana Pimentel et al. Glycine intracerebroventricular administration disrupts mitochondrial energy homeostasis in cerebral cortex and striatum of young rats . In: Neurotoxicity research, New York Vol. 24, no. 4 (Nov. 2013), p. 502-511.
[000970014]
Rosa, Mateus Struecker da et al. Redox homeostasis is compromised in vivo by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase defi ciency in rat cerebral cortex and liver . In: Free radical
research, [London] Vol. 47, no. 12 (Dec. 2013), p. 1066-1075 , il. [000932377]
Santos, André Quincozes dos et al. Resveratrol protects C6 astrocyte cell line against hydrogen
peroxide-induced oxidative stress through heme oxygenase 1 . In: PLoS ONE, San Francisco Vol. 8, no. 5 (May 2013), e64372 [10 p.].
[000875705]
Seminotti, Bianca et al. Disruption of brain redox homeostasis in glutaryl-CoA dehydrogenase deficient mice treated with high dietary lysine supplementation . In: Molecular genetics and metabolism, Orlando Vol. 108, no. 1 (Jan. 2013), p. 30-39.
[000874628]
Tonin, Anelise Miotti et al. Long-chain 3-hydroxy fatty acids accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial trifunctional protein deficiencies uncouple oxidative phosphorylation in heart mitochondria . In: Journal of Bioenergetics and Biomembranes, New York Vol. 45, no. 1-2 (Feb. 2013), p. 47-57.
[000874603]
Zanatta, Angela et al. Neurochemical evidence that the metabolites accumulating in 3-methylcrotonyl-CoA carboxylase deficiency induce oxidative damage in cerebral cortex of young rats . In: Cellular and molecular neurobiology, New York Vol. 33, no. 1 (Jan. 2013), p. 137-146.
Artigo publicado em periódico não indexado estrangeiro
[000942539]
Pires, Ricardo Flores et al. Niemann-Pick type c disease : clinical characterization of a cohort of brazilian patients. In: Latin american journal of human genetics, Colombia Vol. 2, n. 1 (2014), p. 8-15 , il.
Dissertação
[000992574]
Cecatto, Cristiane. Efeitos de ácidos graxos hidroxilados de cadeia longa acumulados nas deficiências da 3-hidroxiacil-CoA desidrogenase de cadeia longa e da proteína trifuncional mitocondrial sobre a homeostase energética mitocondrial nos músculos cardíaco e esquelético de ratos jovens . 2016. 101 f. , il. Dissertação (mestrado) - Universidade Federal do Rio Grande do Sul. Instituto de Ciências Básicas da Saúde. Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Porto Alegre, BR-RS, 2016. Orient.: Wajner, Moacir. [000974479]
Borges, Clarissa Günther. Efeitos da administração in vivo do ácido fitânico sobre parâmetros de homeostase redox em cerebelo de ratos jovens . 2015. 87 f. , il. Dissertação (mestrado) - Universidade Federal do Rio Grande do Sul. Instituto de Ciências Básicas da Saúde. Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Porto Alegre, BR-RS, 2015. Orient.: Wajner, Moacir.
[000968660]
Hickmann, Fernanda Hermes. Efeitos dos ácidos graxos hidroxilados de cadeia longa acumulados na deficiência da desidrogenase de hidroxiacil-CoA de cadeia longa sobre parâmetros de bioenergética mitocondrial em fígado de ratos . 2015. [82] f. , il. Dissertação (mestrado) - Universidade Federal do Rio Grande do Sul. Instituto de Ciências Básicas da Saúde. Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Porto Alegre, BR-RS, 2015. Orient.: Wajner, Moacir.
[000991548]
Rosa, Mateus Struecker da. Efeitos da administração intracerebral dos principais metabólitos acumulados nas acidúrias D-2-hidroxiglutárica e L-2-hidroxiglutárica sobre a homeostase redox e histopatologia no estriado e cerebelo de ratos jovens . 2015. [114] f. , il. Dissertação (mestrado) - Universidade Federal do Rio Grande do Sul. Instituto de Ciências Básicas da Saúde. Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Porto Alegre, BR-RS, 2015. Orient.: Wajner, Moacir.
Tese
[000988192]
Zanatta, Angela. Investigação dos efeitos da ornitina e da homocitrulina sobre as homeostases energética e redox em cerebelo e cultura de astrócitos de córtex cerebral de ratos . 2016. [106] f. , il. Tese (doutorado) -Universidade Federal do Rio Grande do Sul. Instituto de Ciências Básicas da Saúde. Programa de
Pós-Graduação em Ciências Biológicas: Bioquímica, Porto Alegre, BR-RS, 2016. Orient.: Wajner, Moacir. [000965997]
Fernandes, Carolina Gonçalves. Efeitos dos principais ácidos orgânicos acumulados na acidúria
3-hidroxi-3-metilglutárica sobre a homeostase redox, a resposta inflamatória e a fosforilação de proteínas do citoesqueleto em córtex cerebral e estriado de ratos jovens . 2015. [184] f. , il. Tese (doutorado) - Universidade Federal do Rio Grande do Sul. Instituto de Ciências Básicas da Saúde. Programa de Pós-Graduação em
Ciências Biológicas: Bioquímica, Porto Alegre, BR-RS, 2015. Orient.: Wajner, Moacir. Co-Orient.: Pureur, Regina Pessoa.
[000915893]
Amaral, Alexandre Umpierrez. Avaliação da homeostase energética em vários tecidos e histopatologia
cerebral em camundongos nocaute para a enzima glutaril-CoA desidrogenase . 2014. [116] f. Tese (doutorado) - Universidade Federal do Rio Grande do Sul. Instituto de Ciências Básicas da Saúde. Programa de
Pós-Graduação em Ciências Biológicas: Bioquímica, Porto Alegre, BR-RS, 2014. Orient.: Wajner, Moacir. [000939215]
Pierozan, Paula. Efeito do ácido quinolínico sobre a homeostase do citoesqueleto de cérebro de ratos jovens : ênfase nas vias de sinalização, aspectos neuroquímicos, histológicos e morfológicos do dano celular. 2014. [219] f. , il. Tese (doutorado) - Universidade Federal do Rio Grande do Sul. Instituto de Ciências Básicas da Saúde. Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Porto Alegre, BR-RS, 2014. Orient.: Pureur, Regina Pessoa. Co-Orient.: Wajner, Moacir.
[000915901]
Seminotti, Bianca. Estudos sobre a homeostase redox em um modelo animal de acidemia glutárica tipo I . 2014. [134] f. , il. Tese (doutorado) - Universidade Federal do Rio Grande do Sul. Instituto de Ciências Básicas da Saúde. Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Porto Alegre, BR-RS, 2014. Orient.: Wajner, Moacir.
[000917308]
Tonin, Anelise Miotti. Biochemical studies on the physiopathology of MCAD, LCHAD and MTP deficiencies in human fibroblasts and rodent tissues : evidence of disruption of redox and energy homeostasis by
accumulated metabolites. 2014. [136] f. , il. Tese (doutorado) - Universidade Federal do Rio Grande do Sul. Instituto de Ciências Básicas da Saúde. Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Porto Alegre, BR-RS, 2014. Orient.: Wajner, Moacir. Co-Orient.: Gregersen, Niels.
Trabalho de conclusão de curso de graduação
[000934285]
Borges, Clarissa Günther. Avaliação dos efeitos in vitro do ácido hexacosanóico sobre parâmetros de estresse oxidativo em córtex cerebral de ratos jovens . 2013. 55 f. , il. Trabalho de conclusão(graduação)
-Universidade Federal do Rio Grande do Sul. Instituto de Ciências Básicas da Saúde, Porto Alegre, BR-RS, 2013. Orient.: Wajner, Moacir.
[000905115]
Cecatto, Cristiane. Efeitos do ácido etilmalônico sobre a homeostase energética em mitocôndrias de ratos jovens na presença de cálcio . 2013. 33f. Trabalho de conclusão(graduação) - Universidade Federal do Rio Grande do Sul. Faculdade de Farmácia, Porto Alegre, BR-RS, 2013. Orient.: Wajner, Moacir. Co-Orient.: Amaral, Alexandre Umpierrez.
Trabalho publicado em anais de evento realizado fora do país
[000974477]
Guerreiro, Gilian Batista Balbueno et al. Evaluation of the L-carnitine role on the urinary parameters of oxidative stress inmaple syrup urine disease patients . In: Journal of inherited metabolic disease, Lancaster Vol. 38, Suppl. 1 (Sep. 2015), p. S157-S158, resumo P-246 , digital.
[000974503]
Jacques, Carlos Eduardo Diaz et al. Oxidative stress in patientswith 3-hydroxy-3-methylglutaric aciduria and the role of L-carnitine therapy . In: Journal of inherited metabolic disease, Lancaster Vol. 38, Suppl. 1 (Sep. 2015), p. S169, resumo P-278 , digital.
[000975874]
Lorea, Claudia Fernandes et al. Case report : atypical juvenile parkinsonism and basal ganglia calcifications due to HSD 10 disease. In: Journal of inherited metabolic disease, Lancaster Vol. 38, Suppl. 1 (Sep. 2015), p. S210, resumo P-384.
[000974504]
Marchetti, Desirèe Padilha et al. Protective effect of antioxidants on DNA damage in leukocytes from
1 (Sep. 2015), p. S452, resumo P-236 , digital. [000974432]
Mescka, Caroline Paula et al. L-carnitine supplementation decreases DNA damage in treated MSUD patients . In: Journal of inherited metabolic disease, Lancaster Vol. 38, Suppl. 1 (Sep. 2015), p. S139-S140, resumo P-205 , digital.
[000986131]
Poswar, F. et al. Propionic acidemia and humoral immune deficiency . In: Journal of inborn errors of metabolism and screening, Santiago p. 29, resumo PO 49.
[000975901]
Souza, Camila Furtado de et al. Propionic acidemia and humoral immune deficiency . In: Journal of inherited metabolic disease, Lancaster Vol. 38, Suppl. 1 (Sep. 2015), p. S353, resumo A-028.
[000900760]
Amaral, Alexandre Umpierrez et al. Na+, K+-ATPase activity is reduced in cerebral cortex of glutaryl-CoA dehydrogenase deficient mice : a possible mechanism for brain injury in glutaric acidurua type I. In: Journal of Inherited Metabolic Disease, Lancaster Vol. 36, supl. 2 (Sep. 2013), p. S166, Resumo P-199.
[000900465]
Amaral, Alexandre Umpierrez et al. Ethylmalonic acid impairs mitochondrial succinate and malate uptake in rat brain mitochondria . In: Journal of neuroscience research, New York Vol. 91, no. 8 (Aug. 2013), p. 1096. [000900922]
Busanello, Estela Natacha Brandt et al. Mitochondrial oxidative phosphorylation and synaptic membrane Na+,K+-ATPase activity are strongly compromised by pristanic acid in cerebellum of rats . In: Journal of Inherited Metabolic Disease, Lancaster Vol. 36, supl. 2 (Sep. 2013), p. S242, Resumo P-494.
[000900910]
Busanello, Estela Natacha Brandt et al. Alterations of glutamate binding and uptake in striatum from glutaryl-CoA dehydrogenase deficient mice : the role of glutaric acid. In: Journal of Inherited Metabolic Disease, Lancaster Vol. 36, supl. 2 (Sep. 2013), p. S168, Resumo P-207.
Busanello, Estela Natacha Brandt et al. Disruption of bioenergetics by phytanic acid in cerebellum of young rats . In: Journal of neuroscience research, New York Vol. 91, no. 8 (Aug. 2013), p. 1098-1099.
[000900744]
Grings, Mateus et al. Sulfite and thiosulfate compromise brain mitochondrial energy homeostasis . In: Journal of Inherited Metabolic Disease, Lancaster Vol. 36, supl. 2 (Sep. 2013), p. S149, Resumo P-132.
[000900887]
Lagranha, Valeska Lizzi et al. Increased glutamate transporter gene expression in cerebral cortex and striatum from Gcdh-/- mice . In: Journal of Inherited Metabolic Disease, Lancaster Vol. 36, supl. 2 (Sep. 2013), p. S168, Resumo P-205.
[000900893]
Lagranha, Valeska Lizzi et al. Overexpression of glutamate receptors in cerebral cortex and striatum of Gcdh-/- mice : possible implications for the neuropathology of glutaric acidemia type I. In: Journal of Inherited Metabolic Disease, Lancaster Vol. 36, supl. 2 (Sep. 2013), p. S168, Resumo P-206.
[000900872]
Leipnitz, Guilhian et al. Acute administration of 3-hydroxy-3-methylglutaric and 3-methylglutaric acids induces oxidative stress in cerebral cortex and liver of young rats : a possible pathomechanism of brain and liver dysfunction. In: Journal of Inherited Metabolic Disease, Lancaster Vol. 36, supl. 2 (Sep. 2013), p. S167, Resumo P-203.
[000900751]
Moura, Alana Pimentel et al. Mk-801 and antioxidants prevent glycine-induced bioenergetic dysfunction in rat brain : potential novel adjuvant therapies for nonketotic hyperglycinemia. In: Journal of Inherited Metabolic Disease, Lancaster Vol. 36, supl. 2 (Sep. 2013), p. S153-154, Resumo P-149.
[000884529]
Viegas, Carolina Maso et al. In vivo disruption of redox homeostasis caused by the synergistic action of methylmalonic acid and ammonia in brain of young rats . Vol. 125, suppl. 1, p. 255-256 , resumo PTW08-45. [000900861]
Viegas, Carolina Maso et al. Methylmalonic acid and ammonia act synergistically in vivo causing a redox imbalance in cerebral cortex and striatum of young rats . In: Journal of Inherited Metabolic Disease, Lancaster Vol. 36, supl. 2 (Sep. 2013), p. S166-S167, Resumo P-201.
[000900735]
Zanatta, Angela et al. Evidence that ornithine and homocitrulline disturb redox homeostasis in cerebellum of young rats in vivo . In: Journal of Inherited Metabolic Disease, Lancaster Vol. 36, supl. 2 (Sep. 2013), p. S140, Resumo P-096.
Trabalho publicado em anais de evento realizado no país
[000982373]
Lopes, Ana Paula de Abreu et al. Evidência experimental que um comprometimento na bioenergética
mitocondrial não está diretamente envolvido com o dano cerebral apresentado por camundongos nocautes para a glutaril-CoA desidrogenase submetidos a uma sobrecarga de lisina . In: Clinical and biomedical research. Porto Alegre Vol. 35, supl. (set. 2015), p. 48, resumo P3247.
[000990094]
Mescka, Caroline Paula et al. L-carnitine supplementation decreases dna damage in treated MSUD patients . In: Anais p. 31.
[000942380]
Faverzani, Jéssica Lamberty et al. Evidência de dano ao DNA em leucócitos periféricos de pacientes
homocistinúricos . In: Clinical and biomedical research. Porto Alegre Vol. 34, supl. (set. 2014), p. 34, resumo 516.
[000942441]
Vianna, Kawoana Trautman et al. Indução de estresse oxidativo em cérebro de camundongos deficientes para a enzima glutarilcoa desidrogenase submetidos a uma sobrecarga aguda de lisina . In: Clinical and biomedical research. Porto Alegre Vol. 34, supl. (set. 2014), p. 37, resumo 721.
[000899625]
Hammerschmidt, Tatiane Grazieli et al. Evidência experimental de estresse oxidativo em plasma de pacientes com homocistinúria devido à deficiência de cistationina-β-sintase . In: Revista HCPA. Porto Alegre Vol. 33, supl. (ago. 2013), p. 76, [Resumo] 29091. Orient.: Vargas, Carmen Regla.
