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. Author manuscript; available in PMC: 2015 Apr 30.
Published in final edited form as: Sci Transl Med. 2015 Feb 25;7(276):276ra26. doi: 10.1126/scitranslmed.3010101

Fig 6. Filipin-staining of marginal gyrus of cerebral cortex.

Fig 6

Untreated 24-week-old NPC cats (B) showed storage of cholesterol (visualized as white cytoplasmic labeling within cells) in the marginal gyrus of the cerebral cortex; no cholesterol storage was observed in healthy control animals (A). Cats receiving 1000 mg/kg SC HPβCD showed no reduction in cholesterol staining (C). Cats receiving 8000 mg/kg SC HPβCD (D) showed substantially less cholesterol staining than did untreated NPC cats. Indeed, the example shown is from a 37-week-old cat that had received SC HPβCD until 24 weeks of age at which time it was discontinued. 24-week-old cats receiving 30 mg or greater IC HPβCD presymptomatically showed the greatest amelioration of the cholesterol storage defect (E, H). In contrast, treatment of NPC cats with 3.8 mg IC HPβCD (G) or 120 mg IC HPβCD administered post-symptomatically (F) revealed little effect on stored cholesterol in cats at 24 weeks of age, while both 30 mg and 120 mg HPβCD administered presymptomatically showed substantial reductions in cholesterol storage. Scale bar = 100 microns.