Figure 3.
scAAV9.pT-MecP2.CLN8 Treatment Prevents Storage Material Accumulation in Cln8mnd Mice
(A–D) As measured by immunohistochemistry, scAAV9.pT-MecP2.CLN8 treatment significantly prevents autofluorescent storage material (ASM; A and B) and mitochondrial ATP synthase subunit C accumulation (SubC; C and D) in the somatosensory cortex (S1BF; A and C) and thalamus (VPM/VPL; B and D) from 2 through 24 months of age. Images are representative of 8 months of age. Mean ± SEM. Each time point was analyzed independently by either a one-way ANOVA, Fisher’s LSD test (2–8 months), or unpaired t test (10–24 months) (n = 8–72 images/treatment group, biological n = 4/sex/genotype). Detailed sample Ns are described in Table S2. ∗∗∗p < 0.001, ∗∗∗∗p < 0.0001.