Fig. 4.

Neurodegenerative changes affecting cerebellar Purkinje cells. Calbindin-stained sections of cerebellum from wild-type (Wt), NPC1, NPC1;NPC2, and NPC2 mutant mice at (A) 7 and (B) 11 wk of age. (C) A calbindin-stained Purkinje cell in an NPC2 hypomorph mouse cerebellum showing neurodegenerative changes (dendritic and axonal swellings, upper and lower arrows, respectively) typical of all three disease models. (D) Parvalbumin–immunoreactive axonal enlargements (spheroids) in the deep white matter of a cerebellar folium (arrows) of an NPC2 hypomorph mouse that are typical for all three models. Nissl counterstain. Mice were in a mixed C57BL6/129SvEv/BALBc background. [Bars = 500 μm(A), 1,100 μm(B), and 80 μm(D).]