OPA1 and autosomal dominant optic atrophy: An OPA1 gene mutation, B6;C3-Opa1(Q285STOP), which models autosomal dominant optic atrophy, was generated in the mouse.9 The homozygous mutation is embryonic lethal, whereas the heterozygous mutation is associated with visual dysfunction and structural changes in the murine retina and optic nerve beginning at 12 months.9 The animals looked vigorous and appeared healthy.