Altered redox status in Cuban patients with spinocerebellar ataxia type 2

Abstract

Spinocerebellar ataxia 2 (SCA2) is a clinically, pathologically and genetically neurodegenerative disorder. The number of clinical assays in these patients is limited because, among other factors, a lack of biomarkers for the assessment of the main clinical and genetic features of the disease and the evaluation of therapeutic options. The aim of this study was to investigate an extensive array of redox status indexes: glutathione (GSH), malondialdehyde (MDA), peroxidation potential, superoxide dismutase (SOD), ferric reducing ability of plasma (FRAP), catalase (CAT), total hydroperoxide (TH) and advanced oxidation protein products (AOPP) by spectophotometric techniques in relation to electrophysiogical markers in-Cubans SCA2 patients compared to those of healthy aged-gender matched control. The Pearson correlation between progression markers and oxidative stress indexes were assessed too. Nineteen SCA2 patients’ and 19 healthy subjects were recruited. Significant differences (P<0.05) in PP, FRAP, GSH, SOD, AOPP and TH in SCA2 values were found relatively to the control group. The grade of oxidative reaction was evaluated as moderate to severe in almost markers for the studied group. Pearson significant correlation was found between PPLatMOS and FRAP-Lat. These results contribute both to the evidences that oxidative stress occurs during spinocerebellar ataxia type 2 and its useful for the integral management of patients.