Fig. 2.

NPC1 protein abundance in normal and NPC fibroblasts after treatment with MG132 15 μM or glycerol 10% for 24 h. Representative western blot analysis of NPC1 protein expression in normal and NPC fibroblasts cell lines treated with MG132 for 24 h with 15 μM (panel a) or glycerol 10% (panel c). The intensity of the NPC1 signals were normalized against actin. The NPC1 protein content in NPC1 fibroblasts was expressed as a percentage of the NPC1 protein content found in vehicle treated cells (panels b and d). Data are means ± SD of 3 independent experiments (*p < 0.05). Treatment of NPC cells with MG132 15 μM or glycerol significant increase the content of NPC1 mutant protein