Search Results - "Ribas, Graziela S."

l-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders by Ribas, Graziela S., Vargas, Carmen R., Wajner, Moacir

“…In recent years increasing evidence has emerged suggesting that oxidative stress is involved in the pathophysiology of a number of inherited metabolic…”
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The effect of bone marrow transplantation on oxidative stress in X-linked adrenoleukodystrophy by Rockenbach, Francieli J., Deon, Marion, Marchese, Daiane P., Manfredini, Vanusa, Mescka, Caroline, Ribas, Graziela S., Habekost, Clarissa T., Castro, Claudio G., Jardim, Laura B., Vargas, Carmen R.

“…Oxidative stress plays an important role in the pathophysiology of neurodegenerative diseases, including X-linked adrenoleukodystrophy (X-ALD). In the present…”
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Oxidative Stress in Phenylketonuria: What is the Evidence? by Ribas, Graziela S., Sitta, Angela, Wajner, Moacir, Vargas, Carmen R.

“…Phenylketonuria (PKU) is an inborn error of amino acid metabolism caused by severe deficiency of phenylalanine hydroxylase activity, leading to the…”
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Globotriaosylceramide is correlated with oxidative stress and inflammation in Fabry patients treated with enzyme replacement therapy by Biancini, Giovana B., Vanzin, Camila S., Rodrigues, Daiane B., Deon, Marion, Ribas, Graziela S., Barschak, Alethéa G., Manfredini, Vanusa, Netto, Cristina B.O., Jardim, Laura B., Giugliani, Roberto, Vargas, Carmen R.

“…Fabry disease is an X-linked inborn error of glycosphingolipid catabolism due to deficient activity of α-galactosidase A that leads to accumulation of the…”
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Urinary biomarkers of oxidative stress and plasmatic inflammatory profile in phenylketonuric treated patients by Deon, Marion, Sitta, Angela, Faverzani, Jessica L., Guerreiro, Gillian B., Donida, Bruna, Marchetti, Desirèe P., Mescka, Caroline P., Ribas, Graziela S., Coitinho, Adriana S., Wajner, Moacir, Vargas, Carmen R.

“…•Urine from PKU treated patients presented high lipid and protein oxidative damage.•The decreased urinary antioxidant defenses from PKU treated patients was…”
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Neurological Damage in MSUD: The Role of Oxidative Stress by Sitta, Angela, Ribas, Graziela S., Mescka, Caroline P., Barschak, Alethéa G., Wajner, Moacir, Vargas, Carmen R.

“…Maple syrup urine disease (MSUD) is a metabolic disease caused by a deficiency in the branched-chain α-keto acid dehydrogenase complex, leading to the…”
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Oxidative stress in Niemann-Pick type C patients: a protective role of N-butyl-deoxynojirimycin therapy by Ribas, Graziela S., Pires, Ricardo, Coelho, Janice Carneiro, Rodrigues, Daiane, Mescka, Caroline Paula, Vanzin, Camila S., Biancini, Giovana B., Negretto, Giovanna, Wayhs, Carlos A.Y., Wajner, Moacir, Vargas, Carmen R.

“…► Oxidative stress has been demonstrated in NPC1 disease models. ► N-butyl-deoxynojirimycin is the only approved therapy for NPC1 patients. ► We measured…”
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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 by Vanzin, Camila Simioni, Mescka, Caroline Paula, Donida, Bruna, Hammerschimidt, Tatiane Grazieli, Ribas, Graziela S., Kolling, Janaína, Scherer, Emilene B., Vilarinho, Laura, Nogueira, Célia, Coitinho, Adriana Simon, Wajner, Moacir, Wyse, Angela T. S., Vargas, Carmen Regla

“…Cystathionine-β-synthase (CBS) deficiency is the main cause of homocystinuria. Homocysteine (Hcy), methionine, and other metabolites of Hcy accumulate in the…”
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Prevention by l-carnitine of DNA damage induced by propionic and l-methylmalonic acids in human peripheral leukocytes in vitro by Ribas, Graziela S., Manfredini, Vanusa, de Marco, Maria Gilda, Vieira, Rosana B., Wayhs, Carlos Y., Vanzin, Camila S., Biancini, Giovana B., Wajner, Moacir, Vargas, Carmen R.

“…Propionic acidemia (PAemia) and methylmalonic acidemia (MMAemia) are inborn errors of propionate metabolism characterized by the accumulation of, respectively,…”
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Oxidative Stress Parameters in Urine from Patients with Disorders of Propionate Metabolism: a Beneficial Effect of l-Carnitine Supplementation by Ribas, Graziela S., Biancini, Giovana B., Mescka, Caroline, Wayhs, Carlos Y., Sitta, Angela, Wajner, Moacir, Vargas, Carmen R.

“…Propionic (PA) and methylmalonic (MMA) acidurias are inherited disorders caused by deficiency of propionyl-CoA carboxylase and methylmalonyl-CoA mutase,…”
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Experimental evidence for protein oxidative damage and altered antioxidant defense in patients with medium-chain acyl-CoA dehydrogenase deficiency by Derks, Terry G. J., Touw, Catharina M. L., Ribas, Graziela S., Biancini, Giovana B., Vanzin, Camila S., Negretto, Giovanna, Mescka, Caroline P., Reijngoud, Dirk Jan, Smit, G. Peter A., Wajner, Moacir, Vargas, Carmen R.

“…The objective of this study was to test whether macromolecule oxidative damage and altered enzymatic antioxidative defenses occur in patients with medium-chain…”
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Reduction of lipid and protein damage in patients with disorders of propionate metabolism under treatment: a possible protective role of l‐carnitine supplementation by Ribas, Graziela S., Manfredini, Vanusa, Mari, Jurema F., Wayhs, Carlos Y., Vanzin, Camila S., Biancini, Giovana B., Sitta, Angela, Deon, Marion, Wajner, Moacir, Vargas, Carmen R.

“…Disorders of propionate metabolism are autosomal recessive diseases clinically characterized by acute metabolic crises in the neonatal period and long‐term…”
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Protective effect of n-acetyl-L-cysteine and rosuvastatin against oxidative stress in fibroblasts from asymptomatic patients with X-ALD: a preliminary study by Marchetti, Desirèe P., Ribas, Graziela O. S., Donida, Bruna, Brito, Verônica, Moura, Dinara J., Coelho, Daniella M., Machado, Andryele Z., Kessler, Rejane G., Coitinho, Adriana S., Vargas, Carmen R.

“…Introduction: Several studies in the literature have evaluated the role of oxidative stress and adjuvant therapies for X-linked adrenoleukodystrophy (X-ALD)…”
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Reduction of butyrylcholinesterase activity in plasma from patients with disorders of propionate metabolism is prevented by treatment with L-carnitine and protein restriction by Ribas, Graziela S., Scherer, Emilene B., Ferreira, Andrea G., Schmitz, Felipe, Wyse, Angela T., Rodrigues, Daiane, Nascimento, Sabrina, Garcia, Solange C., Wajner, Moacir, Vargas, Carmen R.

“…We investigated the relationship between butyrylcholinesterase (BuChE) activity and lipid oxidative damage in patients with disorders of propionate metabolism,…”
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Effects of insulin and clonazepam on DNA damage in diabetic rats submitted to the forced swimming test by Wayhs, Carlos Alberto Yasin, Manfredini, Vanusa, Sitta, Angela, Deon, Marion, Ribas, Graziela S., Vanzin, Camila S., Biancini, Giovana B., Nin, Maurício S., Barros, Helena M.T., Vargas, Carmen Regla

“…Diabetes mellitus (DM) is a chronic hyperglycemic state. DM may be associated with moderate cognitive deficits and neurophysiologic/structural changes in the…”
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