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Figure 8 | Orphanet Journal of Rare Diseases

Figure 8

From: Visualization of cholesterol deposits in lysosomes of Niemann-Pick type C fibroblasts using recombinant perfringolysin O

Figure 8

Immunoelectron microscopy analysis of GST-PFO staining in NPC and healthy fibroblasts. Ultrathin sections of NPC cells (A, B) or control healthy fibroblasts (C) were subjected to post-embedding immunogold labeling with GST-PFO followed by anti-GST-biotin and anti-biotin-gold antibodies (6 nm) together with rabbit anti-LAMP-1 and anti-rabbit-gold antibodies (10 nm). GST-PFO decorates material accumulated in vesicles of NPC cells (arrows in A, B) while LAMP-1 is visualized mainly at the borders of vesicles in NPC and control fibroblasts (semicircles in A and C).

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