This drug is a synthetic benzoquinone and Coenzyme Q10 analogue that acts as both a mitochondrial electron carrier and as an antioxidant. It is primarily used in the treatment of LHON. Idebenone shows promise for slowing the progression of OPA1-ADOA, and may even provide some restoration of visual function by helping to preserve ATP production and reduce oxidative damage to RGCs. Further studies are needed to evaluate the efficacy in ADOA. Here, OPA1 is linked to autosomal dominant optic atrophy.