Fig. 3.
Npc1−/− mice display reduced granule neuron density in the EGL that is rescued by the HPBCD treatment. (A,C) Cerebellar sections of PN11, PN14 Npc1+/+, and Npc1−/− mice, either untreated or treated with a single injection of HPBCD at PN7, were stained with Hoechst 33258. Representative sections are shown in the figure. Higher magnification fields of EGL base or crown of lobules II and X are shown on the right of low magnification fields. No major differences were detected at PN11. At PN14 however, the EGL of both anterior and posterior lobules of Npc1−/− mice was thinner than that of age-matched Npc1+/+, while HPBCD treatment fully rescued this phenotype. (B,D) Histograms represent granule neuron densities (mean ± SEM of all sections examined; 4–5 mice/group; 3–4 sections/mouse) determined in 100 μm2 regions of the crowns of the anterior (I–V) and posterior (VI–X) lobules of Npc1+/+ (empty bars), Npc1−/− (full bars) and HPBCD-treated Npc1−/− (dashed bars) mice. Asterisks indicate statistically significant differences (*p < 0.05; **p < 0.005). Scale bars indicate 250 μm (panels) and 50 μm (inserts).