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Chronic Cyclodextrin Treatment of Murine Niemann-Pick C Disease Ameliorates Neuronal Cholesterol and Glycosphingolipid Storage and Disease Progression

Figure 1

Combination treatment using NB-DNJ and allopregnanolone/CD in Npc1−/− mice.

(A) Average weight over time for each treatment group shown for males and females separately. (B) Survival of each treatment group. Median survival of Npc1−/− mice: no treatment, 79 days; CD (weekly), 118 days; Miglustat (daily), 123 days; Allopregnanolone/CD (weekly), 146 days; Combination therapy (Miglustat + Allopregnanolone/CD), 152 days.

Figure 1

doi: https://doi.org/10.1371/journal.pone.0006951.g001