While the exact function of Prominin-1 remains unclear, its role in maintaining retinal homeostasis is evident, as mutations in PROM1 have been linked to retinal degeneration in humans with a variety of retinal phenotypes, including autosomal recessive retinitis pigmentosa [12, 13] with macular atrophy [14], autosomal recessive cone-rod dystrophy [15–17], autosomal dominant cone-rod dystrophy and macular phenotypes [18], such as Stargardt-like [19] and bull’s eye maculopathies [20]. This evidence concerns the gene PROM1 and autosomal recessive cone rod dystrophy.