Figure 8.

FLX ameliorates the neurological hallmarks of MPS-IIIA mice

(A) Representative images of immunoblot analysis of p62 in brain protein extracts from vehicle and FLX-treated MPS-IIIA mice. The plot shows the densitometry of the p62 band normalized to actin as mean values ±SEMs (n = 5 per group). t test, ∗∗p < 0.01 versus MPS-IIIA. (B) Representative immunostaining of phopsho-α-synuclein marker (pS129 α-syn) in brain parietal cortex sections from WT and MPS-IIIA mice that were FLX treated compared with the vehicle group. Nuclei were stained with hematoxylin II (blue). The plot shows the mean percentage of p-129-α-synuclein area on the total area ±SEMs (n = 4 per group). ANOVA test, ∗∗p < 0.01 versus WT vehicle; ⋅⋅p < 0.01 versus MPS-IIIA vehicle. (C) Representative images of the immunoblot analysis of ubiquitin in brain protein extracts from WT and MPS-IIIA vehicle and FLX-treated mice. The plot shows the densitometry of polyubiquitinated proteins normalized to actin as mean values ±SEMs (n = 6 per group). ANOVA test, ∗∗p < 0.01 versus WT vehicle; ⋅p < 0.05 versus MPS-IIIA vehicle. (D) FLX-treated WT (n = 9) and MPS-IIIA (n = 9) and relative control vehicle-treated WT (n = 7) and MPS-IIIA (n = 9) were tested at 8 months of age in the fear contextual conditioning test. Unequal post hoc analysis, ∗p < 0.05 versus train; ⋅p < 0.05 versus MPS-IIIA.