Affiliations: Division of Biochemistry and Genetics, Institute of Child Health, London, UK
Note: [] Corresponding author: P.R. Muller: Division of Biochemistry and Genetics Institute of Child Health, 30 Guilford Street, London, WC1N 1EH, UK. Fax: +44 171 404 6191; e-mail: D.Muller@ich.ucl.ac.uk
Abstract: The purpose of this study was to investigate the hypothesis that the gene for Friedreich's ataxia (FA) may be involved in the metabolism of α-tocopherol (vitamin E) or in other antioxidant systems. Concentrations of α-tocopherol, parameters of endogenous lipid peroxidation and susceptibility to in vitro oxidative stress were measured in neural tissues from four patients with FA and four controls. In general there were no significant differences in any of the parameters studied, although the brainstem whole homogenate from the FA patients was significantly (p < 0.02) less susceptible to in vitro oxidative stress than control material. These results, therefore, do not support the suggestion that abnormal metabolism of α-tocopherol or increased oxidative stress is involved in the aetiology of FA.
