Contrasting the reduced NRF2 expression documented in FRDA tissues, NRF2 was significantly activated in both leukocytes and fibroblasts of the two asymptomatic compound heterozygous I-2 and II-2 (Figure 3), suggesting that the occurrence of an endogenous stimulation of this transcription factor in these subjects might translate into protective and preventive effects on the symptomatology. The gene discussed is NFE2L2; the disease is Friedreich ataxia.