Increasing evidence suggests that iron-mediated oxidative stress might underlie the
development of neurodegeneration in Friedreich's ataxia (FRDA), an autosomal recessive
ataxia caused by decreased expression of frataxin, a protein implicated in iron metabolism.
In this study, we demonstrate that, in fibroblasts of patients with FRDA, the cellular redox
equilibrium is shifted toward more protein-bound glutathione. Furthermore, we found that
actin is glutathionylated, probably as a result of the accumulation of reactive oxygen species …