Pathogenic mutations in OPA1 trigger Autosomal Dominant Optic Atrophy (ADOA, MIM#165500) which causes blindness due to Retinal Ganglion Cells (RGCs) death (Delettre et al., 2000; Olichon et al., 2003; Amati-Bonneau et al., 2008). The gene discussed is OPA1; the disease is autosomal dominant optic atrophy.