Chronic Cyclodextrin Treatment of Murine Niemann-Pick C Disease Ameliorates Neuronal Cholesterol and Glycosphingolipid Storage and Disease Progression (original) (raw)
Figure 7
Free sphingosine concentrations in NPC disease following CD treatment.
(A) Biochemical analysis of sphingosine in brain of untreated and CD-treated Npc1−/− and WT mice revealed that chronic CD treatment reduced sphingosine concentrations in affected mice, even when treatment was initiated post-weaning. Npc2−/− mice exhibited a similar trend, although the effect was less pronounced. (B) Biochemical analysis of liver from untreated and CD-treated Npc1−/− and Npc2−/− mice showed that with chronic CD treatment, sphingosine accumulation was reduced to levels near those found in WT.