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Provedor de dados:  Genet. Mol. Biol.
País:  Brazil
Título:  Oxidative stress assessment by glutathione peroxidase activity and glutathione levels in response to selenium supplementation in patients with Mucopolysaccharidosis I, II and VI
Autores:  Oliveira-Silva,José Araújo de
Yamamoto,Joyce Umbelino Pinto
Oliveira,Renata Bernardes de
Monteiro,Vaneisse Cristina Lima
Frangipani,Beatriz Jurkiewcz
Kyosen,Sandra Obikawa
Martins,Ana Maria
D’Almeida,Vânia
Data:  2019-03-01
Ano:  2019
Palavras-chave:  Mucopolysaccharidosis
Oxidative stress
Selenium
Glutathione peroxidase
Resumo:  Abstract We assessed levels of plasma selenium (Se), selenoproteins and their change after Se supplementation in patients with mucopolysaccharidosis (MPS) types I, II and VI. This was done in a retrospective study of the medical records of 30 patients with MPS I (n=13), MPS II (n=9) and MPS VI (n=8) who were being treated with enzyme replacement therapy. As part of routine nutritional monitoring, Se levels were measured, revealing that 28 patients (93.3%) had values below the normal range. Therefore, they received supplementation for 12 months, and Se was measured after 6 and 12 months. Glutathione peroxidase (GPx) activity, total glutathione (GSHt), oxidized glutathione (GSSG) and reduced glutathione (GSH) were measured at baseline and 6 months after Se supplementation. The mean GSHt at baseline was 7.90 ± 2.36 μmol/g Hb, and after Se supplementation it was 5.76 ± 1.13 μmol/g Hb; GSH/GSSG was 2.3 ± 1.16 at baseline and 0.58 ± 0.38 after supplementation. GPx activity was 16.46 ± 3.31 U/g Hb at baseline and 4.53 ± 4.92 U/g Hb after Se supplementation. The difference was shown to be statistically significant by paired t-test. In conclusion, our study demonstrated that oxidative stress parameters were altered by Se supplementation in patients with MPS I, II and VI who were previously deficient in Se.
Tipo:  Info:eu-repo/semantics/article
Idioma:  Inglês
Identificador:  http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1415-47572019000100001
Editor:  Sociedade Brasileira de Genética
Relação:  10.1590/1678-4685-gmb-2017-0334
Formato:  text/html
Fonte:  Genetics and Molecular Biology v.42 n.1 2019
Direitos:  info:eu-repo/semantics/openAccess
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