Fig. 4.

Retinal imaging and functional assessment of BBS7−/− Case 3 at 3.5 years of age (D, E, F, I, K, M) in comparison with an age-matched unaffected monkey (A, B, C, H, J, L). D) color fundus photo of the BBS7−/− case shows abnormal pigmentation and vasculature. E) FAF imaging shows widespread hyperfluorescence but patchy macular hypofluorescence consistent with RPE atrophy. F) Ultra-wide-field fluorescein angiography shows absence of normal macular RPE screening of choroidal fluorescence and localized transmission defects. G) OCT scan, corresponding to the green line in E, illustrates severe thinning and loss of retinal layers. The lower panels illustrate severely reduced amplitudes and delayed time courses of both the scotopic (I), photopic (K) and flicker (M) ERG in comparison to a representative unaffected monkey (H, J, L).