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Fig. 4 | Orphanet Journal of Rare Diseases

Fig. 4

From: Visual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ß-cyclodextrin

Fig. 4

HPßCD corrects the cholesterol storage defect of Npc1 −/− mice. Brain parasagittal sections of PN75 Npc1 +/+ and Npc1 −/− mice, either sham or HPßCD-treated, were stained with filipin. Representative fields (Additional file 3: Figure S3) of a primary visual cortex (V1), b dorsal lateral geniculate nucleus (dLGN) and c retinal ganglion cells are shown in the figure. Note that the cytoplasmic cholesterol accumulation (arrowheads) is almost fully rescued by HPßCD. RPE: retinal pigment ephitelium; OS: outer segments; ONL: outer nuclear layer; OPL: outer plexiform layer; INL: inner nuclear layer; IPL: inner plexiform layer; GCL: ganglion cell layer. Scale bars indicate 50 μm (a, b) and 25 μm (c)

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172