Degeneration of cone and rod photoreceptor cells. (A) The density of arrestin-positive cone photoreceptor cells was markedly reduced in Ctsd ko retinas at P10 (Ab) when compared with age-matched control retinas (Aa). At P25, mutant retinas were devoid of arrestin-positive cones (Ad; for a control retina, see Ac). (B) Quantitative analyses of mutant (filled bars) and control retinas (open bars) confirmed a significantly reduced density of cones in 10-day-old Ctsd ko mouse retinas, and a progressive loss of cones until P25 (Ba). The number of rows of photoreceptor nuclei was similar in Ctsd ko (filled bars) and control retinas (open bars) at P10, but then decreased rapidly in the mutant retinas with increasing age of the animals (Bb). Each bar represents the mean value (±SEM) of at least 6 animals. n.s., not significant; ***, p < 0.001, two-way ANOVA. cn, control; gcl, ganglion cell layer; inl, inner nuclear layer; ko, knock-out; n.s., not significant; onl, outer nuclear layer; P, postnatal day. Scale bar: 100 µm.