Paramount to the effects of Nrf2 on mitochondrial function, a pivotal clinical trial of omaveloxolone in patients with Friedreich's ataxia was recently completed.[8, 9] Friedreich's ataxia is a disease caused by a deficiency in frataxin, a protein involved in the assembly of iron–sulfur clusters, which leads to increased free radicals, oxidative stress, and disruption of mitochondrial homeostasis primarily in the cerebellum, liver, and heart. This evidence concerns the gene FXN and Friedreich ataxia.