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 Here, COQ4 is linked to Duchenne muscular dystrophy.