Similar neurodegenerative events have been described to occur in Friedreich's ataxia (Pandolfo, 2003), where deficiency of the ISC–iron chaperone, frataxin, causes shortages of the ISC‐dependent respiratory complexes that lead to energy deficiency (Lodi et al, 1999; Hick et al, 2013). Here, FXN is linked to Friedreich ataxia.