Several therapeutic strategies targeting either the primary or secondary defects of FRDA are under development following two main directions: (1) restoration of FXN levels using gene expression modulators, protein stabilizers and gene therapy and (2) alleviation of secondary cellular defects such as mitochondrial functions, iron accumulation, oxidative stress and ferroptosis (Clay et al., 2019; Zesiewicz et al., 2020; Lynch and Farmer, 2021; Ocana-Santero et al., 2021; Pallardó et al., 2021; Yang et al., 2021). Here, FXN is linked to Friedreich ataxia.