“…The hypersensitivity of S-cones in ESCS is due to a greater number of S-cones than the more normally abundant L or M-cones (Hood et al, 1995). In humans, different mutations in NR2E3 have been correlated with ESCS (Haider et al, 2000, Hayashi et al, 2005, Milam et al, 2002, Nakamura et al, 2002, Nakamura et al, 2004, Sharon et al, 2003, Wright et al, 2004, although mutations in NRL have also been correlated with ESCS (Wright et al, 2004). In many of the cases where the NR2E3 gene is mutated, an increased number of S-cones, retinal dysplasia and degeneration was observed (Haider et al, 2000).…”