Search Results - "da Rosa, Mateus Struecker"

Disruption of Brain Redox Homeostasis, Microglia Activation and Neuronal Damage Induced by Intracerebroventricular Administration of S-Adenosylmethionine to Developing Rats by Seminotti, Bianca, Zanatta, Ângela, Ribeiro, Rafael Teixeira, da Rosa, Mateus Struecker, Wyse, Angela T. S., Leipnitz, Guilhian, Wajner, Moacir

“…S-Adenosylmethionine (AdoMet) concentrations are highly elevated in tissues and biological fluids of patients affected by S-adenosylhomocysteine hydrolase…”
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Bezafibrate In Vivo Administration Prevents 3-Methylglutaric Acid-Induced Impairment of Redox Status, Mitochondrial Biogenesis, and Neural Injury in Brain of Developing Rats by da Rosa-Junior, Nevton Teixeira, Parmeggiani, Belisa, da Rosa, Mateus Struecker, Glänzel, Nícolas Manzke, de Moura Alvorcem, Leonardo, Wajner, Moacir, Leipnitz, Guilhian

“…3-Methylglutaric acid (MGA) is an organic acid that accumulates in 3-methylglutaconic (MGTA) and 3-hydroxy-3-methylglutaric (HMGA) acidurias. Patients affected…”
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3-Hydroxy-3-Methylglutaric Acid Impairs Redox and Energy Homeostasis, Mitochondrial Dynamics, and Endoplasmic Reticulum–Mitochondria Crosstalk in Rat Brain by da Rosa, Mateus Struecker, da Rosa-Junior, Nevton Teixeira, Parmeggiani, Belisa, Glänzel, Nícolas Manzke, de Moura Alvorcem, Leonardo, Ribeiro, Rafael Teixeira, Grings, Mateus, Wajner, Moacir, Leipnitz, Guilhian

“…3-Hydroxy-3-methylglutaryl-CoA lyase (HL) deficiency is a neurometabolic disorder characterized by predominant accumulation of 3-hydroxy-3-methylglutaric acid…”
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3-Hydroxy-3-methylglutaric and 3-methylglutaric acids impair redox status and energy production and transfer in rat heart: relevance for the pathophysiology of cardiac dysfunction in 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency by da Rosa, Mateus Struecker, Seminotti, Bianca, Ribeiro, César Augusto João, Parmeggiani, Belisa, Grings, Mateus, Wajner, Moacir, Leipnitz, Guilhian

“…3-Hydroxy-3-methylglutaryl-coenzyme A lyase (HL) deficiency is characterized by tissue accumulation of 3-hydroxy-3-methylglutaric (HMG), and 3-methylglutaric…”
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In vivo intracerebral administration of L-2-hydroxyglutaric acid provokes oxidative stress and histopathological alterations in striatum and cerebellum of adolescent rats by da Rosa, Mateus Struecker, João Ribeiro, César Augusto, Seminotti, Bianca, Teixeira Ribeiro, Rafael, Umpierrez Amaral, Alexandre, de Moura Coelho, Daniella, de Oliveira, Francine Hehn, Leipnitz, Guilhian, Wajner, Moacir

“…Patients affected by L-2-hydroxyglutaric aciduria (L-2-HGA) are biochemically characterized by elevated L-2-hydroxyglutaric acid (L-2-HG) concentrations in…”
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Disruption of Energy Transfer and Redox Status by Sulfite in Hippocampus, Striatum, and Cerebellum of Developing Rats by de Moura Alvorcem, Leonardo, da Rosa, Mateus Struecker, Glänzel, Nícolas Manzke, Parmeggiani, Belisa, Grings, Mateus, Schmitz, Felipe, Wyse, Angela T.S., Wajner, Moacir, Leipnitz, Guilhian

“…Patients with sulfite oxidase (SO) deficiency present severe brain abnormalities, whose pathophysiology is not yet elucidated. We evaluated the effects of…”
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α-Ketoadipic Acid and α-Aminoadipic Acid Cause Disturbance of Glutamatergic Neurotransmission and Induction of Oxidative Stress In Vitro in Brain of Adolescent Rats by da Silva, Janaína Camacho, Amaral, Alexandre Umpierrez, Cecatto, Cristiane, Wajner, Alessandro, dos Santos Godoy, Kálita, Ribeiro, Rafael Teixeira, de Mello Gonçalves, Aline, Zanatta, Ângela, da Rosa, Mateus Struecker, Loureiro, Samanta Oliveira, Vargas, Carmen Regla, Leipnitz, Guilhian, de Souza, Diogo Onofre Gomes, Wajner, Moacir

“…Tissue accumulation of α-ketoadipic (KAA) and α-aminoadipic (AAA) acids is the biochemical hallmark of α-ketoadipic aciduria. This inborn error of metabolism…”
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Acute lysine overload provokes protein oxidative damage and reduction of antioxidant defenses in the brain of infant glutaryl-CoA dehydrogenase deficient mice: A role for oxidative stress in GA I neuropathology by Seminotti, Bianca, Ribeiro, Rafael Teixeira, Amaral, Alexandre Umpierrez, da Rosa, Mateus Struecker, Pereira, Carolina Coffi, Leipnitz, Guilhian, Koeller, David M, Goodman, Stephen, Woontner, Michael, Wajner, Moacir

“…Abstract We evaluated the antioxidant defense system and protein oxidative damage in the brain and liver of 15-day-old GCDH deficient knockout ( Gcdh−/− ) mice…”
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Glycine intrastriatal administration induces lipid and protein oxidative damage and alters the enzymatic antioxidant defenses in rat brain by Seminotti, Bianca, Knebel, Lisiane Aurélio, Fernandes, Carolina Gonçalves, Amaral, Alexandre Umpierrez, da Rosa, Mateus Struecker, Eichler, Paula, Leipnitz, Guilhian, Wajner, Moacir

“…We investigated the effects of in vivo intrastriatal administration of glycine (Gly), which is found at high concentrations in the brain of patients affected…”
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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 by Fernandes, Carolina Gonçalves, da Rosa, Mateus Struecker, Seminotti, Bianca, Pierozan, Paula, Martell, Rafael Wolter, Lagranha, Valeska Lizzi, Busanello, Estela Natacha Brandt, Leipnitz, Guilhian, Wajner, Moacir

“…3-Hydroxy-3-methylglutaryl-CoA lyase (HL) deficiency is a genetic disorder biochemically characterized by predominant accumulation of…”
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Disruption of brain redox homeostasis in glutaryl-CoA dehydrogenase deficient mice treated with high dietary lysine supplementation by Seminotti, Bianca, Amaral, Alexandre Umpierrez, da Rosa, Mateus Struecker, Fernandes, Carolina Gonçalves, Leipnitz, Guilhian, Olivera-Bravo, Silvia, Barbeito, Luis, Ribeiro, César Augusto J., de Souza, Diogo Onofre Gomes, Woontner, Michael, Goodman, Stephen I., Koeller, David M., Wajner, Moacir

“…Deficiency of glutaryl-CoA dehydrogenase (GCDH) activity or glutaric aciduria type I (GA I) is an inherited neurometabolic disorder biochemically characterized…”
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Induction of oxidative stress in brain of glutaryl-CoA dehydrogenase deficient mice by acute lysine administration by Seminotti, Bianca, da Rosa, Mateus Struecker, Fernandes, Carolina Gonçalves, Amaral, Alexandre Umpierrez, Braga, Luisa Macedo, Leipnitz, Guilhian, de Souza, Diogo Onofre Gomes, Woontner, Michael, Koeller, David M., Goodman, Stephen, Wajner, Moacir

“…In the present work we evaluated a variety of indicators of oxidative stress in distinct brain regions (striatum, cerebral cortex and hippocampus), the liver,…”
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Evidence that 3-hydroxy-3-methylglutaric and 3-methylglutaric acids induce DNA damage in rat striatum by da Rosa, Mateus Struecker, Scaini, Giselli, Damiani, Adriani Paganini, Longaretti, Luiza Martins, Pereira, Maiara, Seminotti, Bianca, Zapelini, Hugo Galvane, Schuck, Patrícia Fernanda, Streck, Emílio Luiz, de Andrade, Vanessa Moraes, Wajner, Moacir, Leipnitz, Guilhian

“…3-Hydroxy-3-methylglutaryl-CoA lyase (HL) deficiency is a rare autosomal recessive disorderaffecting the final step of leucine degradation and ketogenesis and…”
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