FIGURE 3.
scAAV9.Mecp2.CLN3 administration attenuates storage material accumulation in Cln3 Δex7/8 mice. AAV9 administration attenuates autofluorescent storage material accumulation in the S1BF (A) and VPM/VPL (B) from 2 to 24 months of age in Cln3 Δex7/8 mice. Similarly, AAV9 administration attenuates the accumulation of a lipofuscin constituent, mitochondrial ATP synthase subunit c (SubC) in the S1BF (C) and VPM/VPL (D) throughout the lifespan of Cln3 Δex7/8 mice. One-way ANOVA, Tukey correction at each time point, as each time point was processed independently. Mean ± SEM. *p < 0.05, **p < 0.01, ***p < 0.001, ****p < 0.0001. Scale Bars = 200 µm, images presented are from 6 months of age. 8 images/region/animal were examined for ASM; 12 images/region/animal were examined for SubC.