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

Figure 8. Dantrolene corrects lipid imbalances in I1061T-NPC1–induced neurons.

Figure 8

Isogenic WT, I1061T, or R934L iNeurons were treated with vehicle (Veh) or dantrolene (dan; 10 μM) for 5 days. (A and B) Cells were stained for TuJ1 (green), and unesterified cholesterol was labeled with filipin (cyan). Filipin intensity/TuJ1 quantified at right. (C) LC-MS analysis of relative unesterified cholesterol (area under the curve [AUC]). (D) Cells were stained for GM2 (green). Nuclei were stained with DAPI (blue). Percentage of cells with GM2 puncta are quantified. (E) LC-MS analysis of relative GM2 (34:1) (AUC). Scale bars: 30 μm. Data are shown as the mean ± SEM from (A) 8, (B and D) 4, or (C and E) 3 independent experiments. **P ≤ 0.01, ***P ≤ 0.001, ****P ≤ 0.0001 by ANOVA with Tukey’s post hoc or t test. (A) F = 17.9, DF = 2; (B) t = 0.4 DF = 6; (C) F = 0.3, DF = 2; (D) F = 46.47, DF = 2; (E) F = 0.5, DF = 2.