In vivo analysis of onset and progression of retinal degeneration in the Nr2e3 mouse model of enhanced S-cone sensitivity syndrome.

The photoreceptor-specific nuclear receptor Nr2e3 is not expressed in Nr2e3 mice, a mouse model of the recessively inherited retinal degeneration enhanced S-cone sensitivity syndrome (ESCS). We characterized in detail C57BL/6J Nr2e3 mice in vivo by fundus photography, optical coherence tomography and fluorescein angiography and, post mortem, by histology and immunohistochemistry. White retinal spots and so-called 'rosettes' first appear at postnatal day (P) 12 in the dorsal retina and reach maximal expansion at P21.