Blood oxidative stress markers in Gaucher disease patients

Blood oxidative stress markers in Gaucher disease patients

Author Roversi, F. M. Google Scholar
Galdieri, L. C. Google Scholar
Grego, BHC Google Scholar
Souza, F. G. Google Scholar
Micheletti, C. Google Scholar
Martins, A. M. Google Scholar
D'Almeida, V Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Abstract Background: Gaucher disease (GD) is the most common glycosphingolipidosis resulting in accumulation of glucoceramide. the most effective treatment for this disease is enzyme replacement therapy (ERT) which involves recombinant enzyme infusion. Enzymatic deficiency in GD patients may induce a cascade of events culminating in secondary effects such as the production of reactive oxygen species (ROS). We investigated the relationship between ROS and GD by analyzing blood oxidative stress markers in GD patients submitted to ERT at different stages during the treatment.Methods: Blood were collected before and just after enzyme infusion. Red blood cell catalase (CAT), glutathione peroxidase (GPx), superoxide dismutase (SOD) and total glutathione (tGSH), and plasma thiobarbituric acid reactive substances (TBARS) were assayed by spectrophotometry. Homocysteine concentrations and related polymorphisms were also studied. Control individuals matched for sex and age were also analyzed.Results: Concentrations of homocysteine and TBARS, and GPx enzyme activity were not different in ERT-treated GD patients. CAT activity was higher while SOD was lower in patients compared to controls. No variations in any of these parameters were found before and just after ERT. Regarding tGSH, a significant increase was observed in GD patients after infusion. Genotypic frequencies studied did not differ from controls or other Brazilian samples.Conclusion: ERT-treated GD patients show an improvement in antioxidant capacity, which is further increased just after recombinant enzyme infusion. (C) 2005 Elsevier B.V. All rights reserved.
Keywords Gaucher disease
enzyme replacement therapy
oxidative stress
free radicals
Language English
Date 2006-02-01
Published in Clinica Chimica Acta. Amsterdam: Elsevier B.V., v. 364, n. 1-2, p. 316-320, 2006.
ISSN 0009-8981 (Sherpa/Romeo, impact factor)
Publisher Elsevier B.V.
Extent 316-320
Access rights Closed access
Type Article
Web of Science ID WOS:000235376800040

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