Oxidative stress assessment by glutathione peroxidase activity and glutathione levels in response to selenium supplementation in patients with Mucopolysaccharidosis I, II and VI

• Published in: Genetics and molecular biology Vol. 42; no. 1; pp. 1 - 8
• Main Authors: 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
• Format: Journal Article
• Language: English
• Published: Brazil Sociedade Brasileira de Genetica 01.01.2019; Sociedade Brasileira de Genética
• Subjects: BIOCHEMISTRY & MOLECULAR BIOLOGY; Dietary supplements; GENETICS & HEREDITY; Glutathione; Glutathione peroxidase; Human and Medical Genetics; Medical records; Mucopolysaccharidosis; Nutrition monitoring; Oxidation; Oxidative stress; Peroxidase; Selenium; Selenoproteins; Statistical analysis; Mucopolysaccharidosis; selenium; oxidative stress; glutathione peroxidase
• ISSN: 1415-4757; 1678-4685; 1678-4685
• DOI: 10.1590/1678-4685-GMB-2017-0334

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.