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. 2020 Jul 15;31(13-14):709–718. doi: 10.1089/hum.2020.038

Figure 3.

Figure 3.

Intravitreal administration of 7m8.CMV.hCLN3 prevents the loss of retinal function and bipolar cells in Cln3Δex7/8 mice. (A) Scotopic ERG b-wave amplitudes of 7m8.CMV.hCLN3-treated mutant mice. Wild type: n = 7–8 eyes, Cln3Δex7/8: n = 8 eyes, 7m8.CMV.hCLN3: n = 10 eyes. Wild-type recordings from Fig. 1 were added as a reference. (B) Scotopic ERG traces of a Cln3Δex7/8 mouse that received treatment in one eye (red) and no treatment in the contralateral eye (black). (C) Confocal images of the midretina from untreated and 7m8.CMV.hCLN3-treated eyes stained for PKCα at 15 months. (D) Counts of PKCα-positive cells in the midretina at 15 months. Data are presented as mean ± SD and analyzed by (A) two-way ANOVA with Bonferroni test and (D) one-way ANOVA with Kruskal–Wallis test (*p < 0.05, ***p < 0.001). Scale bar: 25 μm. hCLN3, human CLN3.