Fig. 2

Abnormal morphology of HPS1 patient-specific CPM-isolated lung bud organoids (C-LBOs). A Overview of the generation of C-LBOs from iPSCs. B Chronological observation of C-LBOs. (Scale bars: 1000 μm) C Quantification of organoid size. Data are presented as mean ± SEM (on Day 7, n = 4 organoids from 4 independent experiments; on Day 21 and Day 35, n = 12 organoids from 4 independent experiments). Two-way ANOVA with Sidak’s multiple comparisons test: **P < 0.01, ***P < 0.001. D Expression levels of cytokines (TGFB1 and IL11), pan-epithelial cell markers (EPCAM and CDH1), transcription factors (SNAI1 and SNAI2), and mesenchymal cell markers (cytoskeleton, ACTA2 and VIM; cell surface, PDGFRB and THY1; extra cellular matrix, COL1A1 and FN1) in C-LBOs evaluated by qRT-PCR, normalized to that of human adult lung. Data are presented as mean ± SEM (n = 6 from 5 independent experiments). Two-way ANOVA with Sidak’s multiple comparisons test: *P < 0.05, **P < 0.01. n.s.: not significance. E, F Morphologies of HPS1 patient-specific C-LBOs on Day 35, treated with 10 μM SB431542 from Day 0 to Day 35. (Scale bars: 1000 μm) Data are presented as mean ± SEM (n = 11 or 12 organoids from 1 independent experiment). One-way ANOVA with Tukey’s multiple comparisons test: ***P < 0.001. n.s.: not significance