Notably, Raxone®, a synthetic CoQ10 analogue was approved by the European Medicines Agency (EMA) to treat LHON in 2015 [99,100], and LUMEVOQ®, an allotopic ND4 gene replacement therapy for the most common LHON mtDNA mutation (m.11778G>A; MT-ND4), received a temporary authorisation for use in France in 2021 (EURETINA) (Phase III RESCUE, REVERSE, REFLECT, GenSight Biologics; [28,101,102]). Here, MT-ND4 is linked to Leber hereditary optic neuropathy.