Fig. 4.
Npc1−/− mice display reduced number of proliferating granule neurons that is rescued by the HPBCD treatment. (A) Histograms represent the number of BrdU-positive cells (mean ± SEM; 4–5 mice/group; 3–4 sections/mouse) determined in 100 μm2 regions corresponding to the bases and 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). Note that the number of BrdU-positive GNs was significantly reduced in both crowns and fissures of the anterior cerebellar lobules of PN12 and PN14 Npc1−/− mice compared to age-matched Npc1+/+ mice. (B) A representative field showing BrdU-positive cells (red) of fissure between lobules I and II of PN12 Npc1+/+ (left), Npc1−/− (middle) and HPBCD-treated Npc1−/− mice (right). Note that HPBCD treatment re-established the normal number of proliferating cells. Scale bar indicates 50 μm.