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. Author manuscript; available in PMC: 2011 May 1.
Published in final edited form as: Traffic. 2010 Feb 22;11(5):601–615. doi: 10.1111/j.1600-0854.2010.01046.x

Figure 8.

Figure 8

Decreased LSO cholesterol accumulation and increased acid SMase activity are achieved in NPC1 human fibroblasts by increasing amounts of rhASM. WT (GM5659) and NPC (GM03123) Fbs were incubated with 0, 0.2 and 1.8 μg/mL rhASM for 24 h. To remove surface-bound label, the cells were further incubated for 15 min with growth medium without the enzyme. The cells were washed thoroughly with PBS and either lysed and assayed for acid SMase activity (A) or fixed and stained with filipin for imaging and quantification (B). Each data point in plot (A) is representative of three wells in an experiment, p < 0.05. Plot (B) represents LSO ratios normalized to the untreated NPC Fb GM01323 values (average of 16–20 images from two independent experiments ±SEM), p < 0.005.