Figure 3.

Phenotypic characterization of heterozygous and homozygous KI mice. (A-B) Retinal structure of KI mice. Retinal integrity of 11-month WT/KI and KI/KI mice was monitored by both in vivo OCT imaging (A) and H&E staining of retina methacrylate sections (B). While there are no differences in retinal structure between WT/KI (middle panel in B) and their WT siblings (left panel in B), KI/KI mice show the presence of vacuoles in the outer segment layer (arrow in right panel of 2B). Scale bar=50 μm. (C-D) Visual function analysis show that both the rod (C) and cone (D) functions of the WT/KI and KI/KI mice are comparable to the WT sibling controls; n=10–12 for each genotype group. (E-F) HPLC analysis of the retinoid content in the KI mice following 24-hour dark adaptation. The retinae of the WT/KI and KI/KI mice contain similar levels of 11-cis retinal as those of the WT (E). However, the retinyl ester levels in the KI/KI mice eyes are significantly increased compared to those in the WT eyes (F); n=3 for each genotype group in the HPLC analysis. INL: inner nuclear layer; ONL: outer nuclear layer; OS: outer segment; and RPE: retinal pigment epithelium.