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. 2022 Dec 8;7(23):e160308. doi: 10.1172/jci.insight.160308

Figure 2. Mouse I1061T-NPC1 retains function.

Figure 2

(A and B) Lysates from primary mouse fibroblasts and human Hap1 cells with WT-NPC1, I1061T-NPC1, or Null-NPC1 were analyzed by Western blot for total NPC1 and quantified. (C) Cholesterol storage was measured by filipin (cyan) staining in WT-NPC1, I1061T-NPC1, and NPC1-null human Hap1 cells or mouse fibroblasts. WGA (orange) was used to label plasma membranes. Scale bar: 40 μm. Filipin intensity was normalized relative to WT and quantified. Data are shown as the mean ± SEM from 3 to 4 independent experiments. *P ≤ 0.05, **P ≤ 0.01, ***P ≤ 0.001, ****P ≤ 0.0001 by ANOVA with Tukey’s post hoc. (A and B) F = 282.0, 43.5, 282.0; df = 2. (C) F = 23.4; 666.0; df = 2.