Treatment with CoQ10 or its derivatives (eg, idebenone) has shown some beneficial effects in other recessive multisystemic ataxias (eg, TXN2),31 with at least mixed effects in Friedreich ataxia,32 primary CoQ10 deficiencies with neurological features (eg, COQ2, COQ4, or COQ7),26, 33, 34, 35 and respiratory dysfunction in Duchenne muscular dystrophy,36 and is already approved for Leber’s hereditary optic neuropathy.37 The gene discussed is COQ4; the disease is Friedreich ataxia.