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. 2018 Apr 12;27(12):2101–2112. doi: 10.1093/hmg/ddy117

Figure 3.

Figure 3.

Alexidine treatment induces NPC1 expression. (A) Western blot following 72 h drug treatment in fibroblasts from indicated genotypes, Anti-acetylated tubulin demonstrates appropriate HDACi activity. (B) Quantification of (A), n = 3 biological replicates, mean ± SD, ****P < 0.0001 one-way ANOVA followed by Tukey’s test. (C, D) EndoH digestion of NPC1 reveals an increase in both mature and immature NPC1 following alexidine treatment. NPC1I1061T/I1061T(C) or normal human fibroblasts (D) were plated and treated 24 h later with DMSO or alexidine. After 72 h, cells were harvested then subjected to Endo Hf or vehicle treatment. Western blot and normalized quantification for NPC1 in mutant (C) and normal (D) cell lysates. Open triangles mark Endo Hf resistant (Endo HR) species. Closed triangles mark Endo Hf sensitive (Endo HS) species. ****P < 0.0001, ***P < 0.001, **P < 0.01 versus glycospecies matched DMSO, two-way ANOVA followed by Dunnett’s test. (E) NPC1 expression by TaqMan qRT-PCR in indicated cell lines following 48 h drug treatment. n = 3 biological replicates, mean ± SD, ****P < 0.0001 versus DMSO, $versus Control alexidine two-way ANOVA followed by Dunnett’s test.