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. 2008 Sep 5;283(36):24584–24593. doi: 10.1074/jbc.M803715200

FIGURE 3.

FIGURE 3.

NPC1 is required for the efficient clearance of amines that accumulate in lysosomes. A, Western blot analysis of NPC1 expression in normal fibroblasts before and after transfection with NPC1 siRNA. Actin expression was used as a sample loading control. B, quantitative evaluation of NR release shows NPC1+/+ fibroblasts (○) clear NR more efficiently than NPC1-/- fibroblasts (•) or NPC1+/+ fibroblasts pretreated with NPC1 siRNA (▾). Each time point represents the mean ± S.D. of 3–10 individual measurements. C, fluorescence micrographs of the lysosomal vital stain LTR showing the enhanced clearance in NPC1+/+ fibroblasts relative to the NPC1-/- fibroblasts. D, lysosomal clearance of LTR is not impaired in lysosomal storage disorder fibroblasts mucolipidosis type IV (MLIV) and Sandhoff disease. All LTR images are representative of data collected from at least three independent experiments.