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Figure 6 | Orphanet Journal of Rare Diseases

Figure 6

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

Figure 6

GST-PFO labeling of NPC cells colocalizes with LBPA. NPC (A-B”) and healthy control fibroblasts (C-C”) were fixed, permeabilized with 0.05% Triton X-100 (A-A”) or 0.05% digitonin (B-C”) and labeled with GST-PFO and anti-LBPA antibody. (A, B, C) GST-PFO labeling, (A’, B’, C’) LBPA localization, (A”, B”, C”) merged images. TX, Triton X-100, DiT, digitonin. Scale bar, 20 μm.

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