Figure 7.

NAC treatment restores normal GSH levels in Npc1−/− mice. From 4 to 8 weeks of age, untreated Npc1−/− mice (open circles) had significantly decreased GSH levels compared with Npc1+/+ mice (triangles) in liver (A) and brain (B) (P < 0.05). When NAC was administered to Npc1−/− mice from 4 to 8 weeks of age (filled circles), significant increases in both liver (A) and brain (B) GSH were seen relative to untreated Npc1−/− mice (P < 0.01). These increased GSH levels achieved in NAC-treated Npc1−/− mice were statistically similar to those of Npc1+/+ (P ≥ 0.08). NAC-treated Npc1−/− mice received NAC at 250 mg/kg NAC beginning at 4 weeks of age (P28). Significance determined by two-way ANOVA. n = 4–5 for each treatment group.