Figure 8

Disease mechanisms in C57BL/6J Nr2e3rd7/rd7 mice. Schematic drawing of disease mechanisms in the C57BL/6J Nr2e3rd7/rd7 mouse model of ESCS. (A) Early postnatal steps in ‘rosette’ formation. At P12, ‘rosettes’ appear dorsally (yellow), at P13 a pan-retinal expansion towards the periphery is observed (orange), and at P21 ‘rosettes’ have expanded to the far periphery. Scale bar: 250 µM. (B) Inflammatory cell population in young and aged C57BL/6J Nr2e3rd7/rd7 retinas. At P21 and P28 activated microglial cells (light green) migrate into the rosettes and few monocyte/macrophage-derived cells (dark green) are present in the retina. In aged retinas, the outer nuclear layer is thinning and subretinal macrophages (dark green) are regularly spaced in the subretinal space. RPE: retinal pigment epithelium; OS: outer segments; ONL: outer nuclear layer; OPL: outer plexiform layer; INL: inner nuclear layer; IPL: inner plexiform layer; GCL: ganglion cell layer.