Chronic Cyclodextrin Treatment of Murine Niemann-Pick C Disease Ameliorates Neuronal Cholesterol and Glycosphingolipid Storage and Disease Progression (original) (raw)
Figure 4
Chronic allopregnanolone and chronic CD treatment studies in Npc1−/− mice.
(A) Survival of untreated and allopregnanolone-treated Npc1−/− mice using different vehicles for allopregnanolone. Median survival of Npc1−/− mice: DMSO, 84 days; Allo/DMSO, 83 days; Corn Oil, 81 days; Allo/Corn Oil, 88 days; 5% CD, 114 days; Allo/5% CD, 121 days. (B) Average weight over time for untreated and chronically CD-treated Npc1−/− and WT mice. Weights of untreated and CD-treated WTs were averaged for each gender as there was no significant difference between treatments (p<0.8125). (C) Survival of untreated and CD-treated Npc1−/− and WT mice showing effects of different start times. Median survival of Npc1−/− mice: no treatment, 83 days; CD (every other day, start at P7), 185 days; CD (every other day, start at P21), 149 days. Treatment initiated at P7 appeared more efficacious, although lifespan was not significantly longer when compared to treatment initiated at P21 (p<0.1870).