Friedreich’s Ataxia (FRDA) is an early-onset autosomal recessive disease with an incidence of 1:50000, caused by severely reduced levels of frataxin, a mitochondrial protein involved in iron–sulfur cluster synthesis, iron transfer, and antioxidant defense (Romeo et al., 1983; Dürr et al., 1996; Santos et al., 2010; Vaubel and Isaya, 2013). Here, FXN is linked to Friedreich ataxia.