Search Results - "de Moura Coelho, Daniella"

Oxidative damage in glutaric aciduria type I patients and the protective effects of l‐carnitine treatment by Guerreiro, Gilian, Faverzani, Jéssica, Jacques, Carlos Eduardo Diaz, Marchetti, Desirèe P., Sitta, Angela, Moura Coelho, Daniella, Kayser, Aline, Kok, Fernando, Athayde, Larissa, Manfredini, Vanusa, Wajner, Moacir, Vargas, Carmen Regla

“…The deficiency of the enzyme glutaryl‐CoA dehydrogenase, known as glutaric acidemia type I (GA‐I), leads to the accumulation of glutaric acid (GA) and…”
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Clinical, biochemical and molecular findings of 24 Brazilian patients with glutaric acidemia type 1: 4 novel mutations in the GCDH gene by Sitta, Angela, Guerreiro, Gilian, de Moura Coelho, Daniella, da Rocha, Vitoria Volfart, dos Reis, Bianca Gomes, Sousa, Carmen, Vilarinho, Laura, Wajner, Moacir, Vargas, Carmen Regla

“…Glutaric aciduria type 1 (GA-1) is a rare but treatable inherited disease caused by deficiency of glutaryl-CoA dehydrogenase activity due to GCDH gene…”
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Inflammatory profile in X‐linked adrenoleukodystrophy patients: Understanding disease progression by Marchetti, Desirèe Padilha, Donida, Bruna, Jacques, Carlos Eduardo, Deon, Marion, Hauschild, Tatiane Cristina, Koehler‐Santos, Patricia, de Moura Coelho, Daniella, Coitinho, Adriana Simon, Jardim, Laura Bannach, Vargas, Carmen Regla

“…X‐linked adrenoleukodystrophy (X‐ALD) is an inherited disease characterized by progressive inflammatory demyelization in the brain, adrenal insufficiency, and…”
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L-carnitine protects DNA oxidative damage induced by phenylalanine and its keto acid derivatives in neural cells: a possible pathomechanism and adjuvant therapy for brain injury in phenylketonuria by Faverzani, Jéssica Lamberty, Steinmetz, Aline, Deon, Marion, Marchetti, Desirèe Padilha, Guerreiro, Gilian, Sitta, Angela, de Moura Coelho, Daniella, Lopes, Franciele Fatima, Nascimento, Leopoldo Vinicius Martins, Steffens, Luiza, Henn, Jeferson Gustavo, Ferro, Matheus Bernardes, Brito, Verônica Bidinotto, Wajner, Moacir, Moura, Dinara Jaqueline, Vargas, Carmen Regla

“…Although phenylalanine (Phe) is known to be neurotoxic in phenylketonuria (PKU), its exact pathogenetic mechanisms of brain damage are still poorly known…”
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Oxidative damage in mitochondrial fatty acids oxidation disorders patients and the in vitro effect of l-carnitine on DNA damage induced by the accumulated metabolites by de Moraes, Maira Silmara, Guerreiro, Gilian, Sitta, Angela, de Moura Coelho, Daniella, Manfredini, Vanusa, Wajner, Moacir, Vargas, Carmen Regla

“…The mitochondrial fatty acids oxidation disorders (FAOD) are inherited metabolic disorders (IMD) characterized by the accumulation of fatty acids of different…”
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Acute administration of 5-oxoproline induces oxidative damage to lipids and proteins and impairs antioxidant defenses in cerebral cortex and cerebellum of young rats by Pederzolli, Carolina Didonet, Mescka, Caroline Paula, Zandoná, Bernardo Remuzzi, de Moura Coelho, Daniella, Sgaravatti, Ângela Malysz, Sgarbi, Mirian Bonaldi, de Souza Wyse, Angela Terezinha, Duval Wannmacher, Clóvis Milton, Wajner, Moacir, Vargas, Carmen Regla, Dutra-Filho, Carlos Severo

“…5-Oxoproline accumulates in glutathione synthetase deficiency, an autossomic recessive inherited disorder clinically characterized by hemolytic anemia,…”
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Evidence That Long-Term Treatment Prevents Tissue Oxidative Damage in Patients With Inherited Disorders of the Propionate Pathway by Dos Reis, Bianca Gomes, Becker, Graziela Schmitt, Marchetti, Desiree Padilha, Coelho, Daniella de Moura, Sitta, Angela, Wajner, Moacir, Vargas, Carmen Regla

“…Propionic and methylmalonic acidemias (PAcidemia and MMAcidemia, respectively) are genetic disorders clinically characterized by metabolic decompensation…”
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Prevention by L-carnitine of DNA damage induced by 3-hydroxy-3-methylglutaric and 3-methylglutaric acids and experimental evidence of lipid and DNA damage in patients with 3-hydroxy-3-methylglutaric aciduria by Delgado, Camila Aguilar, Balbueno Guerreiro, Gilian Batista, Diaz Jacques, Carlos Eduardo, de Moura Coelho, Daniella, Sitta, Angela, Manfredini, Vanusa, Wajner, Moacir, Vargas, Carmen Regla

“…3-hydroxy-3-methylglutaric aciduria (HMGA) is an inherited disorder of the leucine catabolic pathway in which occurs a deficiency of the…”
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Increased cytokine levels induced by high phenylalanine concentrations in late diagnosis PKU patients compared to early diagnosis: Anti‐inflammatory effect of L‐carnitine by Faverzani, Jéssica Lamberty, Hammerschmidt, Tatiane Grazieli, Mescka, Caroline Paula, Guerreiro, Gilian, Lopes, Franciele Fatima, Delgado, Camila Aguilar, Moura Coelho, Daniella, Sitta, Angela, Deon, Marion, Wajner, Moacir, Vargas, Carmen Regla

“…Phenylketonuria (PKU) was the first genetic disease to have an effective therapy, which consists of phenylalanine intake restriction. However, there are…”
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Selective Screening of Fatty Acids Oxidation Defects and Organic Acidemias by Liquid Chromatography/tandem Mass Spectrometry Acylcarnitine Analysis in Brazilian Patients by Vargas, Carmen Regla, Ribas, Graziela Schmitt, da Silva, Janine Machado, Sitta, Angela, Deon, Marion, de Moura Coelho, Daniella, Wajner, Moacir

“…Inborn errors of metabolism (IEM) are diseases which can lead to accumulation of toxic metabolites in the organism. To investigate, by selective screening,…”
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Experimental evidence of oxidative stress in patients with l-2-hydroxyglutaric aciduria and that l-carnitine attenuates in vitro DNA damage caused by d-2-hydroxyglutaric and l-2-hydroxyglutaric acids by Rodrigues, Daiane Grigolo Bardemaker, de Moura Coelho, Daniella, Sitta, Ângela, Jacques, Carlos Eduardo Diaz, Hauschild, Tatiane, Manfredini, Vanusa, Bakkali, Abdellatif, Struys, Eduard A., Jakobs, Cornelis, Wajner, Moacir, Vargas, Carmen Regla

“…d-2-hydroxyglutaric (D-2-HGA) and l-2-hydroxyglutaric (L-2-HGA) acidurias are rare neurometabolic disorders biochemically characterized by increased levels of…”
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Urinary biomarkers of oxidative damage in Maple syrup urine disease: The l-carnitine role by Guerreiro, Gilian, Mescka, Caroline Paula, Sitta, Angela, Donida, Bruna, Marchetti, Desirèe, Hammerschmidt, Tatiane, Faverzani, Jessica, Coelho, Daniella de Moura, Wajner, Moacir, Dutra-Filho, Carlos Severo, Vargas, Carmen Regla

“…•Maple syrup urine disease (MSUD) patients have l-carnitine (l-car) deficiency.•There is damage to proteins and lipids in urine and a deficient antioxidant…”
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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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Preliminary results of PBA-loaded nanoparticles development and the effect on oxidative stress and neuroinflammation in rats submitted to a chemically induced chronic model of MSUD by Mescka, Caroline Paula, de Moura Coelho, Daniella, Sitta, Angela, Catarino, Felipe, Donida, Bruna, Rosa, Andrea Pereira, Gonzalez, Esteban Alberto, Pinheiro, Camila Vieira, Poletto, Fernanda, Baldo, Guilherme, Dutra-Filho, Carlos Severo, Vargas, Carmen Regla

“…Maple syrup urine disease (MSUD) is a genetic disorder that leads the accumulation of branched-chain amino acids (BCAA) leucine (Leu), isoleucine, valine and…”
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Increased oxidative stress in patients with 3-hydroxy-3-methylglutaric aciduria by dos Santos Mello, Mariana, Ribas, Graziela Schmitt, Wayhs, Carlos Alberto Yasin, Hammerschmidt, Tatiane, Guerreiro, Gilian Batista Balbueno, Favenzani, Jéssica Lamberty, Sitta, Ângela, de Moura Coelho, Daniella, Wajner, Moacir, Vargas, Carmen Regla

“…3-hydroxy-3-methylglutaric aciduria (HMGA; OMIM 246450) is a rare autosomal recessive disorder, caused by the deficiency of 3-hydroxy-3-methylglutaryl-CoA…”
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L-Carnitine supplementation decreases DNA damage in treated MSUD patients by Mescka, Caroline Paula, Guerreiro, Gilian, Hammerschmidt, Tatiane, Faverzani, Jéssica, de Moura Coelho, Daniella, Mandredini, Vanusa, Wayhs, Carlos Alberto Yasin, Wajner, Moacir, Dutra-Filho, Carlos Severo, Vargas, Carmen Regla

“…Maple syrup urine disease (MSUD) is an inherited disorder caused by severe deficient activity of the branched-chain α-keto acid dehydrogenase complex involved…”
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Effect of in vivo administration of ethylmalonic acid on energy metabolism in rat tissues by FERREIRA, Gustavo Da C, ROTH ANDRE, Karina, SCHUCK, Patricia Fernanda, VIEGAS, Carolina M, TONIN, Anelise, DE MOURA COELHO, Daniella, WYSE, Angela T. S, WANNMACHER, Clovis M. D, VARGAS, Carmen R, WAJNER, Moacir

“…High concentrations of ethylmalonic acid (EMA) occur in tissues and biological fluids of patients affected by deficiency of short-chain acyl-CoA dehydrogenase…”
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Monitoring of Phenylalanine Levels in Patients with Phenylketonuria Using Dried Blood Spots: a Comparison of Two Methods by Sebastião, Fernanda Medeiros, Burin, Maira Graeff, Civallero, Gabriel, Tirelli, Kristiane Michelin, Sitta, Angela, Coelho, Daniella de Moura, Vargas, Carmen Regla, Wajner, Moacir, Giugliani, Roberto, Bitencourt, Fernanda Hendges de, Schwartz, Ida Vanessa Doederlein

“…Abstract Phenylketonuria (PKU) is caused by deficient activity of phenylalanine hydroxylase (PAH), responsible for the conversion of phenylalanine (Phe) to…”
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Neurodegenerative biomarkers and inflammation in patients with propionic and methylmalonic acidemias: effect of L-carnitine treatment by dos Reis, Bianca Gomes, Becker, Graziela Schmitt, Marchetti, Desirèe Padilha, de Moura Coelho, Daniella, Sitta, Angela, Wajner, Moacir, Vargas, Carmen Regla

“…Propionic and methylmalonic acidemias (PAcidemia and MMAcidemia, respectively) are genetic disorders characterized by acute metabolic decompensation and…”
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Increased peripheral of brain-derived neurotrophic factor levels in phenylketonuric patients treated with l-carnitine by Faverzani, Jéssica Lamberty, Guerreiro, Gilian, Hammerschmidt, Tatiane Grazieli, Lopes, Franciele Fátima, Coelho, Daniella de Moura, Sitta, Angela, Mescka, Caroline Paula, Deon, Marion, Wajner, Moacir, Vargas, Carmen Regla

“…Phenylketonuria (PKU) is the most common inherited metabolic disorders caused by severe deficiency or absence of phenylalanine hydroxylase activity that…”
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