Fig. 1: Loss of CIB2 leads to age-related photoreceptor dysfunction and RPE pathophysiology.

a Schematic of specific constructs used to generate mouse models. b Schematic representation of the multiple retinal layers. ERG a-wave originates from photoreceptors (PR), while b-wave amplitude originates from Müller glia and bipolar cells, and oscillatory potentials come from the amacrine cells. RPE retinal pigment epithelium. c Quantification of scotopic responses from WT (Cib2^WT/WT), Cib2^KO/+, and Cib2^KO/KO mice at indicated ages reveals progressive loss of both a- (left panels) and b-wave (right panels) amplitudes in Cib2-deficient mice. d TEM micrographs of RPE/ outer segment (OS) interface from 8-months-old mice revealed vacuoles (*) and basal infoldings loss specifically in Cib2^KO/+ and Cib2^KO/KO mutants (n = 3 per genotype). Scale bar, 2 μm. e Representative photomicrographs of RPE whole mounts from 9-to-10-months-old mice for denoted genotype showing accumulation of neutral lipid marker BODIPY-493/503 (cyan) in Cib2-deficient mice. Phalloidin (magenta) was used to decorate the actin cytoskeleton. Scale bar, 10 µm. f Quantification of average number (left) or average size (right) of BODIPY-493/503-stained puncta per mouse shown in e. At least three images per mouse (~25 cells/image) were quantified and averages per mouse are shown for WT, Cib2^KO/+, and Cib2^KO/KO mice (n = 3 each). Data presented as mean ± SEM; each data point represents an individual animal. One-way ANOVA and Bonferroni post hoc test, p < 0.05 (*) or <0.001 (***).