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. Author manuscript; available in PMC: 2015 Sep 3.
Published in final edited form as: Cell Rep. 2015 Aug 20;12(9):1385–1390. doi: 10.1016/j.celrep.2015.07.062

Figure 2. Generation of Functional Respiratory Epithelial Cells from CF mutant and gene corrected iPSC.

Figure 2

A) Representative images of CF corrected and CF mutant iPSC differentiation to DE (Day 5); DAPI (Blue) and FOXA2 (Cyan), Scale bar white =100µm, yellow =20µm. B) mRNA expression of DE, AFE and lung epithelial cells markers over the time course of differentiation for wildtype, CF mutant and CF corrected iPSC; data are corrected for internal controls and normalized to wildtype iPSC cDNA (mean ±SEM, n=6–15 from a minimum of 3 experimental replicates). C) Averaged current/voltage plots for baseline (blue), in presence of Forskolin, genistein and isobutylmethyl xanthine (IBMX) (pink) and with the addition of CFTRinh-172 (green).Data represented are mean ±SEM for 0 of 4 patched mutant cells and 3 of 8 patched gene corrected cells. Lower panels are representative current traces from one gene corrected differentiated epithelial cell at baseline, and in the presence of FSK cocktail and CFTRinh-172 (L to R), traces represent 20mV increments from −80 to +80mV. D) Western blot showing the presence of the membrane translocated and glycosylated protein in the wildtype and gene corrected cells (Band B) and the unglycoslylated CFTR band in all 3 cell lines (Band A). WT= wildtype, M = mutant, C= gene corrected.