The most common eye afflictions associated with nDNA mutations are autosomal dominant optic atrophy (ADOA), most frequently due to mutations in the Dynamin-like GTPase OPA1, and autosomal recessive optic atrophy (AROA), which has been mainly associated with mutations in the aconitate hydratase ACO2, or the uncharacterised transmembrane protein TMEM126A (OPA7) 76. Here, OPA1 is linked to autosomal dominant optic atrophy.