Wild-type PC1 promotes ciliary localization of endogenous PC2 in ADPKD hepatoblasts. Wild-type PC1 promotes ciliary localization of endogenous PC2 in ADPKD hepatoblasts. (A) Average number of GFP-positive cells per confluent well of a 24-well plate in untransfected (control) or transfected (GFP-PKD1) populations. (B) Representative images of iPS-derived hepatoblasts from ADPKD patient 3 exogenously expressing GFP-tagged wild-type human PC1 full-length (GFP-PC1 positive) or neighboring untransfected control cells (GFP-PC1 negative) from the same cultures. GFP was detected in the green channel, PC2 was detected in the red channel, and AcT was detected in the far red channel. The merged image was pseudocolored without GFP to highlight colocalization of PC2 and acetylated tubulin. Scale bar, 5 µm. (C) Averaged intensity profiles of line scans through 25 individual cilia from GFP+ or untransfected neighboring cells pooled from four different experiments with hepatoblasts derived from ADPKD patients 2 or 3. Data points represent averaged raw fluorescence intensity values from images taken with identical exposures. Line scans were 16 µm in length. Red dotted line overlay in B illustrates how line scans were drawn. (D) Average PC2/AcT fluorescence ratios for the points at the apex of the curves shown in C. Error bars indicate SEM. P values, t test. Benjamin S. Freedman et al. JASN 2013;24:1571-1586 ©2013 by American Society of Nephrology