Search Results - "Amaral, Alexandre U"

Disturbance of mitochondrial energy homeostasis caused by the metabolites accumulating in LCHAD and MTP deficiencies in rat brain by Tonin, Anelise M., Ferreira, Gustavo C., Grings, Mateus, Viegas, Carolina M., Busanello, Estela N., Amaral, Alexandre U., Zanatta, Ângela, Schuck, Patrícia Fernanda, Wajner, Moacir

“…We investigated the in vitro effects of 3-hydroxydodecanoic (3HDA), 3-hydroxytetradecanoic (3HTA) and 3-hydroxypalmitic (3HPA) acids, which accumulate in…”
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l-Carnitine prevents oxidative stress in striatum of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload by Guerreiro, Gilian, Amaral, Alexandre U., Ribeiro, Rafael Teixeira, Faverzani, Jéssica, Groehs, Ana Carolina, Sitta, Angela, Deon, Marion, Wajner, Moacir, Vargas, Carmen Regla

“…The deficiency of the enzyme glutaryl-CoA dehydrogenase leads to predominant accumulation of glutaric acid (GA) in the organism and is known as glutaric…”
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Deregulation of mitochondrial functions provoked by long‐chain fatty acid accumulating in long‐chain 3‐hydroxyacyl‐CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart – mitochondrial permeability transition pore opening as a potential contributing pathomechanism of cardiac alterations in these disorders by Cecatto, Cristiane, Hickmann, Fernanda H, Rodrigues, Marília D. N, Amaral, Alexandre U, Wajner, Moacir

“…Mitochondrial trifunctional protein and long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiencies are fatty acid oxidation disorders biochemically characterized…”
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Sulfite disrupts brain mitochondrial energy homeostasis and induces mitochondrial permeability transition pore opening via thiol group modification by Grings, Mateus, Moura, Alana P., Amaral, Alexandre U., Parmeggiani, Belisa, Gasparotto, Juciano, Moreira, José C.F., Gelain, Daniel P., Wyse, Angela T.S., Wajner, Moacir, Leipnitz, Guilhian

“…Sulfite oxidase (SO) deficiency is biochemically characterized by the accumulation of sulfite, thiosulfate and S-sulfocysteine in tissues and biological fluids…”
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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 by Tonin, Anelise M., Amaral, Alexandre U., Busanello, Estela N. B., Grings, Mateus, Castilho, Roger F., Wajner, Moacir

“…Cardiomyopathy is a common clinical feature of some inherited disorders of mitochondrial fatty acid β-oxidation including mitochondrial trifunctional protein…”
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Experimental Evidence that Phenylalanine Provokes Oxidative Stress in Hippocampus and Cerebral Cortex of Developing Rats by Fernandes, Carolina G, Leipnitz, Guilhian, Seminotti, Bianca, Amaral, Alexandre U, Zanatta, Ângela, Vargas, Carmen R, Dutra Filho, Carlos S, Wajner, Moacir

“…High levels of phenylalanine (Phe) are the biochemical hallmark of phenylketonuria (PKU), a neurometabolic disorder clinically characterized by severe mental…”
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Neurochemical evidence that phytanic acid induces oxidative damage and reduces the antioxidant defenses in cerebellum and cerebral cortex of rats by Leipnitz, Guilhian, Amaral, Alexandre U., Zanatta, Ângela, Seminotti, Bianca, Fernandes, Carolina G., Knebel, Lisiane A., Vargas, Carmen R., Wajner, Moacir

“…In the present work we investigated the in vitro effects of phytanic acid (Phyt), that accumulates in Refsum disease and other peroxisomal diseases, on…”
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Mechanistic Bases of Neurotoxicity Provoked by Fatty Acids Accumulating in MCAD and LCHAD Deficiencies by Amaral, Alexandre U., Cecatto, Cristiane, da Silva, Janaína C., Wajner, Alessandro, Wajner, Moacir

“…Fatty acid oxidation defects (FAODs) are inherited metabolic disorders caused by deficiency of specific enzyme activities or transport proteins involved in the…”
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Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats by Leipnitz, Guilhian, Seminotti, Bianca, Amaral, Alexandre U., de Bortoli, Giorgia, Solano, Alexandre, Schuck, Patrícia F., Wyse, Ângela T.S., Wannmacher, Clóvis M.D., Latini, Alexandra, Wajner, Moacir

“…3-Methylglutaconic (MGT), 3-methylglutaric (MGA) and occasionally 3-hydroxyisovaleric (OHIVA) acids accumulate in a group of diseases known as…”
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Evidence that 3-hydroxy-3-methylglutaric acid promotes lipid and protein oxidative damage and reduces the nonenzymatic antioxidant defenses in rat cerebral cortex by Leipnitz, Guilhian, Seminotti, Bianca, Haubrich, Josué, Dalcin, Manuela B., Dalcin, Karina B., Solano, Alexandre, de Bortoli, Giorgia, Rosa, Rafael B., Amaral, Alexandre U., Dutra-Filho, Carlos S., Latini, Alexandra, Wajner, Moacir

“…In the present work we investigated the in vitro effect of 3‐hydroxy‐3‐methylglutarate (HMG) that accumulates in 3‐hydroxy‐3‐methylglutaryl‐CoA lyase…”
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Glycine Provokes Lipid Oxidative Damage and Reduces the Antioxidant Defenses in Brain Cortex of Young Rats by Leipnitz, Guilhian, Solano, Alexandre F, Seminotti, Bianca, Amaral, Alexandre U, Fernandes, Carolina G, Beskow, Ana Paula, Dutra Filho, Carlos S, Wajner, Moacir

“…Patients affected by nonketotic hyperglycinemia (NKH) usually present severe neurological symptoms and suffer from acute episodes of intractable seizures with…”
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Evidence that 2-methylacetoacetate induces oxidative stress in rat brain by Leipnitz, Guilhian, Seminotti, Bianca, Amaral, Alexandre U, Fernandes, Carolina G, Dutra-Filho, Carlos S, Wajner, Moacir

“…In the present study we investigated the effects of 2-methylacetoacetate (MAA) and 2-methyl-3-hydroxybutyrate (MHB), the major metabolites accumulating in…”
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Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3‐hydroxy‐3‐methylglutaryl‐CoA lyase deficiency as compared to liver by Leipnitz, Guilhian, Seminotti, Bianca, Fernandes, Carolina G., Amaral, Alexandre U., Beskow, Ana Paula, Silva, Lucila de B., Zanatta, Ângela, Ribeiro, César A.J., Vargas, Carmen R., Wajner, Moacir

“…The present work investigated the in vitro effects of 3‐hydroxy‐3‐methylglutarate, 3‐methylglutarate, 3‐methylglutaconate and 3‐hydroxyisovalerate, which…”
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D-Serine induces lipid and protein oxidative damage and decreases glutathione levels in brain cortex of rats by da Silva, Lucila de Bortoli, Leipnitz, Guilhian, Seminotti, Bianca, Fernandes, Carolina G, Beskow, Ana Paula, Amaral, Alexandre U, Wajner, Moacir

“…Abstract The present work investigated the in vitro effects of d -serine (D-Ser) on important parameters of oxidative stress in cerebral cortex of young rats…”
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Lysine induces lipid and protein damage and decreases reduced glutathione concentrations in brain of young rats by Seminotti, Bianca, Leipnitz, Guilhian, Amaral, Alexandre U., Fernandes, Carolina G., Silva, Lucila de Bortoli da, Tonin, Anelise Miotti, Vargas, Carmen R., Wajner, Moacir

“…The present work investigated the in vitro effects of lysine on important parameters of oxidative stress in cerebral cortex of young rats. Our results show…”
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Induction of oxidative stress by the metabolites accumulating in isovaleric acidemia in brain cortex of young rats by Solano, Alexandre F, Leipnitz, Guilhian, De Bortoli, Giorgia M, Seminotti, Bianca, Amaral, Alexandre U, Fernandes, Carolina G, Latini, Alexandra S, Dutra-Filho, Carlos S, Wajner, Moacir

“…The present work investigated the in vitro effects of isovaleric acid (IVA) and isovalerylglycine (IVG), which accumulate in isovaleric acidemia (IVAcidemia),…”
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Influence of ketone bodies on oxidative stress parameters in brain of developing rats in vitro by Beskow, Ana Paula, Fernandes, Carolina Gonçalves, Leipnitz, Guilhian, da Silva, Lucila de Bortoli, Seminotti, Bianca, Amaral, Alexandre U., Wyse, Angela T. S., Wannmacher, Clóvis M. D., Vargas, Carmen R., Dutra-Filho, Carlos S., Wajner, Moacir

“…Pro-oxidant and antioxidant properties have been found for acetoacetate (AcAc) and β-hydroxybutyrate (BHB) in peripheral tissues. In the present study we…”
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Neurotoxic mechanisms of glyphosate exposure by Limberger, Christian, Roginski, Ana Cristina, Amaral, Alexandre U., Venturin, Gianina Teribele, Wajner, Moacir, daCosta, Jaderson Costa, Zimmer, Eduardo R., Souza, Diogo O., de Souza, Débora Guerini

“…Background Human exposure to pesticides is increasing, especially glyphosate‐based herbicides (GBH). Thus, it is of great interest to characterize whether…”
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