Fig. 6.

Immunohistochemical analysis of retinal disease progression in BBS7−/− macaques Case 1 (4 years) and Case 2 (6 years), and in a 25-year-old BBS7+/− heterozygote with normal retina shown for comparison. A) Retinal pigment epithelium (RPE65 antibody) and cone cells (cone arrestin). B) Müller glia (GFAP) and reactive microglial cells (IBA1). C) Rod bipolar cells (PKCα). For all panels, DAPI indicates nuclear stain. In both cases, posterior retina was significantly more affected with severe atrophy throughout all cell layers. With increasing eccentricity from the macula, the retina appeared more organized with proper lamination, presence of cone photoreceptors, and rod bipolar cells with normal positioning of dendrites and axon terminal boutons. The greatest difference between the two cases was observed in the severity of mid-peripheral retinal degeneration, which was worse in the older animal. White scale bars represent 50 μm.