OPA1 and autosomal dominant optic atrophy: In more recent times, further genes have been associated as relatively frequent cause of ADOA, including proteases indirectly implicated in OPA1 proteolytic processing such as AFG3L2 and SPG7 [111,112], or ACO2, encoding a matrix Krebs’ cycle enzyme [113] and other rarer forms linked to dominant mutations in the OPA3 [114], WFS1 [115], DNM1L [116], and SSBP1 [117,118,119] genes.