Figure 8.

Cone long-term survival in Nphp5^−/− ;Nrl^−/− retina. A–E) Nphp5^−/+;Nrl^−/− (left) and Nphp5^−/−;Nrl^−/− retina sections (right) probed with anti–ML-opsin antibody at P10 (A), P15 (B), 1 mo (C), 2 mo (D), and 3 mo (E) of age. F–I) Nphp5^−/+ (left) and Nphp5^−/− (right) retina sections probed with anti–ML-opsin antibody at P10 (F), P15 (G), 1 mo (H), and 2 mo (I). Note that cone degeneration in Nrl^−/−;Nphp5^−/− retina is slowed and mutant cones still express ML-opsin at 3 mo. Panels A–C were from mice expressing EGFP-CETN2. J) Statistical evaluation ONL thickness at P10, P15, 1 mo, 2 mo, and 3 mo. K) Representative ERG traces of Nphp5^−/−;Nrl^−/− (red), Nphp5^+/− (black), and Nrl^−/− mice (green) at P15 and 1 mo. Nrl^−/− photopic responses are elevated and double-knockout cones are nonfunctional. IS, inner segment; ns, not significant; OPL, outer plexiform layer.