RPE65-knockout (RPE65−/−) mice that had been created from C57BL/6J mice [15] demonstrated slow rod degeneration and rapid cone degeneration [15–17], and can be considered to be a model of human RPE65 gene-associated LCA and RP in humans, although there may be some differences in the course of retinal degeneration between RPE65−/− mice and human patients with RPE65-associated LCA and RP [18]. Here, RPE65 is linked to retinitis pigmentosa 1.