Fig. 8

Oligodendrocyte maturation is impaired in Npc1 ^nmf164 mice. a Western blot analysis of MBP protein expression in cerebella of PN11 and PN15 wt and Npc1 ^nmf164 mice. Histograms indicate the abundance (mean ± SEM) of each isoform determined by densitometry of protein bands obtained in at least 3 independent experiments taking β-actin as internal reference. * p ≤ 0.01, *** p < 0.0001. b Immunostaining with antibodies directed to MBP (brown) shows that PN15 Npc1 ^nmf164 mouse cerebella display a reduction of MBP expression at the level of PC axons and white matter compared to wt littermates. Representative fields of parasagittal sections of lobule III of PN15 wt and Npc1 ^nmf164 mouse cerebella are shown in the Fig.; scale bars: 100 μm. c Immunostaining with antibodies directed to MBP (brown) showing that PN15 Npc1 ^nmf164 mice display a poorer oligodendrocyte differentiation as indicated by the reduced length of MBP-positive processes that typically radiate from oligodendrocyte soma (arrowheads), compared to wt littermates. Representative fields of parasagittal sections of PN15 wt and Npc1 ^nmf164 mouse cerebral cortex (Cx) and splenium of corpus callosum (scc) are shown in the Fig.; scale bars: 100 μm. Higher magnifications are shown in panel C (bottom); scale bars: 20 μm