Fig. 1.

N-PRβ-KO mice show severe retinal structural defects in adult mice.

(a) Schematic representation of the transgenic and mutated alleles of N-PRβ-KO. Exons 4 to 7 of the Pdgfrb gene flanked by two loxP sites are deleted by nlsCre activity driven by the Nestin promoter. (b and c) N-PRβ-KO mice showed vitreous opacity (b, red arrowhead) and detached retina (c) compared with wild-type (WT) mice at 8–12 weeks old. (d) Scoring of retinal deformities. 0, normal cup-shaped retina; 1, distorted cup-shaped retina; 2, detached V-shaped retina or shrunken retina; 3, highly detached V-shaped and very thin peripheral retina or highly shrunken and very thin retina; 4, rudimentary retina; 5, not detected. (e and f) Both male (e) and female (f) N-PRβ-KO mice showed higher deformity scores at 8–12 weeks old than those of WT mice. n = 11–14 male retinas, n = 30–33 female retinas. (g) Typical proliferative membrane on the retinal surface of N-PRβ-KO mice at 10 weeks old. The proliferative membrane includes αSMA-positive cells (red) and blood vessels (indicated by asterisks in magenta). Hoechst staining depicts nuclei (blue). (h) Histology (H&E staining) of the peripheral region of the N-PRβ-KO retina at 10 weeks old. Highly perturbed thin retinas are observed compared to WT retinas. (i and j) N-PRβ-KO showed significantly thinner retinas than those of WT mice for both males (i) and females (j) in the adult stage. n = 5–8 males and females. All values represent means ± SEM. *, p < 0.05; **, p < 0.01; ***, p < 0.001, compared to WT mice at the same time points. Scale bar = 1 mm (c), 100 μm (g and h).