SOD2 and Friedreich ataxia: However, GSH has been observed to remain completely stable despite an increase in GCL expression, raising the possibility that increased GCL expression may not always translate to elevated GSH level [117], In addition, administration of 1 μM EPI-743 decreased lipid peroxidation and rescued the morphological defects of the mitochondria, suggesting restoration of redox homeostasis modulated by ferroptosis-related genes, namely the FXN, NRF2, SOD2, glutathione peroxidase 4 (GPX4), and GCL in fibroblasts derived from patients with FRDA [118].