Moreover, FXN deficiency caused decreased expression NRF2 and its target genes both in vitro in Schwann cells, fibroblasts, and HeLa cells and in vivo in the dorsal root ganglia and cerebellum of YG8R hemizygous mice, an FRDA mouse model that exhibits a mild, late-onset FRDA-like phenotype [21]. Here, FXN is linked to Friedreich ataxia.