Search Results - "Cecatto, Cristiane"

2‐Methylcitric acid impairs glutamate metabolism and induces permeability transition in brain mitochondria by Amaral, Alexandre Umpierrez, Cecatto, Cristiane, Castilho, Roger Frigério, Wajner, Moacir

“…Accumulation of 2‐methylcitric acid (2MCA) is observed in methylmalonic and propionic acidemias, which are clinically characterized by severe neurological…”
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Functional hypoxia reduces mitochondrial calcium uptake by Donnelly, Chris, Komlódi, Timea, Cecatto, Cristiane, Cardoso, Luiza H.D., Compagnion, Anne-Claire, Matera, Alessandro, Tavernari, Daniele, Campiche, Olivier, Paolicelli, Rosa Chiara, Zanou, Nadège, Kayser, Bengt, Gnaiger, Erich, Place, Nicolas

“…Mitochondrial respiration extends beyond ATP generation, with the organelle participating in many cellular and physiological processes. Parallel changes in…”
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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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Stability of mitochondrial respiration medium used in high-resolution respirometry with living and permeabilized cells by Baglivo, Eleonora, Cardoso, Luiza H.D., Cecatto, Cristiane, Gnaiger, Erich

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High-resolution respirometry for measurement of fatty acid β-oxidation in brain mitochondria. A comparative study and application in ischemia and aging by Cardoso, Luiza H.D., Cecatto, Cristiane, Ozola, Melita, Korzh, Stanislava, Zvejniece, Liga, Gukalova, Baiba, Doerrier, Carolina, Dambrova, Maija, Makrecka-Kuka, Marina, Gnaiger, Erich, Liepinsh, Edgars

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Impairment of mitochondrial bioenergetics and permeability transition induction caused by major long-chain fatty acids accumulating in VLCAD deficiency in skeletal muscle as potential pathomechanisms of myopathy by Cecatto, Cristiane, Amaral, Alexandre Umpierrez, Roginski, Ana Cristina, Castilho, Róger Frigério, Wajner, Moacir

“…cis-5-Tetradecenoic (cis-5) and myristic (Myr) acids predominantly accumulate in patients affected by very long-chain acyl-CoA dehydrogenase (VLCAD)…”
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Fatty acid β-oxidation in brain mitochondria: Insights from high-resolution respirometry in mouse, rat and Drosophila brain, ischemia and aging models by Cardoso, Luiza H.D., Cecatto, Cristiane, Ozola, Melita, Korzh, Stanislava, Zvejniece, Liga, Gukalova, Baiba, Doerrier, Carolina, Dambrova, Maija, Makrecka-Kuka, Marina, Gnaiger, Erich, Liepinsh, Edgars

“…Glucose is the main energy source of the brain, yet recent studies demonstrate that fatty acid oxidation (FAO) plays a relevant role in the pathogenesis of…”
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Disturbance of bioenergetics and calcium homeostasis provoked by metabolites accumulating in propionic acidemia in heart mitochondria of developing rats by Roginski, Ana Cristina, Wajner, Alessandro, Cecatto, Cristiane, Wajner, Simone Magagnin, Castilho, Roger Frigério, Wajner, Moacir, Amaral, Alexandre Umpierrez

“…Propionic acidemia is caused by lack of propionyl-CoA carboxylase activity. It is biochemically characterized by accumulation of propionic (PA) and…”
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Disturbance of mitochondrial functions associated with permeability transition pore opening induced by cis-5-tetradecenoic and myristic acids in liver of adolescent rats by Cecatto, Cristiane, Amaral, Alexandre Umpierrez, Wajner, Alessandro, Wajner, Simone Magagnin, Castilho, Roger Frigério, Wajner, Moacir

“…•Cis-5-tetradecenoic (Cis-5) and myristic (Myr) acids accumulate in VLCAD deficiency.•Cis-5 and Myr impair respiration in liver mitochondria and permeabilized…”
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Glycine disrupts myelin, glutamatergic neurotransmission, and redox homeostasis in a neonatal model for non ketotic hyperglycinemia by Parmeggiani, Belisa, Signori, Marian Flores, Cecatto, Cristiane, Frusciante, Marina Rocha, Marcuzzo, Manuela Bianchin, Souza, Débora Guerini, Ribeiro, Rafael Teixeira, Seminotti, Bianca, Gomes de Souza, Diogo Onofre, Ribeiro, César Augusto João, Wajner, Moacir, Leipnitz, Guilhian

“…Non ketotic hyperglycinemia (NKH) is an inborn error of glycine metabolism caused by mutations in the genes encoding glycine cleavage system proteins. Classic…”
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Disruption of Bioenergetics in the Intestine of Wistar Rats Caused by Hydrogen Sulfide and Thiosulfate: A Potential Mechanism of Chronic Hemorrhagic Diarrhea in Ethylmalonic Encephalopathy by Frusciante, Marina Rocha, Signori, Marian Flores, Parmeggiani, Belisa, Grings, Mateus, Pramio, Julia, Cecatto, Cristiane, de Andrade Silveira, Josyane, Aubin, Mariana Rauback, Santos, Larissa Aguiar, Paz, Ana Helena, Wajner, Moacir, Leipnitz, Guilhian

“…Ethylmalonic encephalopathy (EE) is a severe inherited metabolic disorder that causes tissue accumulation of hydrogen sulfide (sulfide) and thiosulfate in…”
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cis-4-Decenoic and decanoic acids impair mitochondrial energy, redox and Ca(2+) homeostasis and induce mitochondrial permeability transition pore opening in rat brain and liver: Possible implications for the pathogenesis of MCAD deficiency by Amaral, Alexandre Umpierrez, Cecatto, Cristiane, da Silva, Janaína Camacho, Wajner, Alessandro, Godoy, Kálita Dos Santos, Ribeiro, Rafael Teixeira, Wajner, Moacir

“…Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is biochemically characterized by tissue accumulation of octanoic (OA), decanoic (DA) and cis-4-decenoic…”
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Experimental evidence that maleic acid markedly compromises glutamate oxidation through inhibition of glutamate dehydrogenase and [alpha]-ketoglutarate dehydrogenase activities in kidney of developing rats by Roginski, Ana Cristina, Cecatto, Cristiane, Wajner, Simone Magagnin, Camera, Fernanda Dal'Maso, Castilho, Roger Frigério, Wajner, Moacir, Amaral, Alexandre Umpierrez

“…Maleic acid (MA), which has been reported to be highly excreted in propionic acidemia (PAcidemia), was demonstrated to cause nephropathy by bioenergetics…”
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Ethylmalonic acid impairs bioenergetics by disturbing succinate and glutamate oxidation and induces mitochondrial permeability transition pore opening in rat cerebellum by Moura Alvorcem, Leonardo, Britto, Renata, Cecatto, Cristiane, Cristina Roginski, Ana, Rohden, Francieli, Nathali Scholl, Juliete, Guma, Fátima C. R., Figueiró, Fabrício, Umpierrez Amaral, Alexandre, Zanatta, Geancarlo, Seminotti, Bianca, Wajner, Moacir, Leipnitz, Guilhian

“…Tissue accumulation and high urinary excretion of ethylmalonic acid (EMA) are found in ethylmalonic encephalopathy (EE), an inherited disorder associated with…”
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Experimental evidence that maleic acid markedly compromises glutamate oxidation through inhibition of glutamate dehydrogenase and α-ketoglutarate dehydrogenase activities in kidney of developing rats by Roginski, Ana Cristina, Cecatto, Cristiane, Wajner, Simone Magagnin, Camera, Fernanda Dal’Maso, Castilho, Roger Frigério, Wajner, Moacir, Amaral, Alexandre Umpierrez

“…Maleic acid (MA), which has been reported to be highly excreted in propionic acidemia (PAcidemia), was demonstrated to cause nephropathy by bioenergetics…”
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High vulnerability of the heart and liver to 3‐hydroxypalmitic acid–induced disruption of mitochondrial functions in intact cell systems by Cecatto, Cristiane, Wajner, Alessandro, Vargas, Carmen Regla, Wajner, Simone Magagnin, Amaral, Alexandre Umpierrez, Wajner, Moacir

“…Patients affected by long‐chain 3‐hydroxyacyl‐CoA dehydrogenase (LCHAD) deficiency predominantly present severe liver and cardiac dysfunction, as well as…”
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Uncoupling, metabolic inhibition and induction of mitochondrial permeability transition in rat liver mitochondria caused by the major long-chain hydroxyl monocarboxylic fatty acids accumulating in LCHAD deficiency by Hickmann, Fernanda Hermes, Cecatto, Cristiane, Kleemann, Daniele, Monteiro, Wagner Oliveira, Castilho, Roger Frigério, Amaral, Alexandre Umpierrez, Wajner, Moacir

“…Patients with long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) deficiency commonly present liver dysfunction whose pathogenesis is unknown. We studied the…”
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Disturbance of mitochondrial functions provoked by the major long-chain 3-hydroxylated fatty acids accumulating in MTP and LCHAD deficiencies in skeletal muscle by Cecatto, Cristiane, Godoy, Kálita Dos Santos, da Silva, Janaína Camacho, Amaral, Alexandre Umpierrez, Wajner, Moacir

“…The pathogenesis of the muscular symptoms and recurrent rhabdomyolysis that are commonly manifested in patients with mitochondrial trifunctional protein (MTP)…”
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cis-4-Decenoic and decanoic acids impair mitochondrial energy, redox and Ca2+ homeostasis and induce mitochondrial permeability transition pore opening in rat brain and liver: Possible implications for the pathogenesis of MCAD deficiency by Amaral, Alexandre Umpierrez, Cecatto, Cristiane, da Silva, Janaína Camacho, Wajner, Alessandro, Godoy, Kálita dos Santos, Ribeiro, Rafael Teixeira, Wajner, Moacir

“…Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is biochemically characterized by tissue accumulation of octanoic (OA), decanoic (DA) and cis-4-decenoic…”
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Metabolite accumulation in VLCAD deficiency markedly disrupts mitochondrial bioenergetics and Ca2+ homeostasis in the heart by Cecatto, Cristiane, Amaral, Alexandre Umpierrez, Silva, Janaína Camacho, Wajner, Alessandro, Schimit, Mariana de Oliveira Vargas, Silva, Lucas Henrique Rodrigues, Wajner, Simone Magagnin, Zanatta, Ângela, Castilho, Roger Frigério, Wajner, Moacir

“…We studied the effects of the major long‐chain fatty acids accumulating in very long‐chain acyl‐CoA dehydrogenase (VLCAD) deficiency, namely…”
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