Forebrain dysmyelination in mice following neuron-specific deletion of <i>Npc1</i>.

<p>(A) Schematic of midline sagittal section of the mouse brain, with the area shown in panel B highlighted by the black rectangle. Illustration is from <a href="http://www.gensat.org" target="_blank">www.gensat.org</a>. (B) (Top three panels) MBP immunofluorescence in forebrain sagittal sections of <i>Npc1^flox/−, Syn1-Cre⁺</i> and control mice at P16, and at 7 & 16 weeks. Bar, 500 µm. Area highlighted by white rectangle is enlarged in the inset in the upper right of each panel. (Bottom panel) FluoroMyelin staining of the corpus callosm of <i>Npc1^flox/−, Syn1-Cre⁺</i> and control mice at 16 weeks. Ctx, cortex; CC, corpus callosum; Hp, hippocampus. Bar, 200 µm. (C) Western blots of myelin-specific proteins and neurofilament protein (NF-200) from brainstem and cerebral cortex homogenates of P16 <i>Npc1^flox/−, Syn1-Cre⁺</i> mice and controls. GAPDH controls for loading. (D) MBP and neurofilament protein (NF) co-staining of P16 <i>Npc1^flox/−, Syn1-Cre⁺</i> and littermate control mice. Ctx, cortex; CC, corpus callosum; Hp, hippocampus. Bar, 200 µm. (E) Electron microscopy of the corpus callosum of P16 <i>Npc1^flox/−, Syn1-Cre</i> and control mice. Bar, 500 nm.