Figure 5.

Ciliary expression of SHH signaling components

Control and INPP5E^D477N/D477N organoids were immunostained with the indicated markers.

(A–H) SMO was expressed in a higher proportion of cilia and at higher levels in INPP5E^D477N/D477N organoids.

(I–P) There were no significant changes either in the proportion of positive cilia or in the expression levels for SUFU.

(Q–X) GLI2 accumulated in mutant cilia.

Statistical data are presented as means ± 95% confidence intervals (CIs); unpaired t tests (D, L, and T) and Mann-Whitney tests (H, P, and X); n = 3 (control) and n = 2 (mutant) lines for (D), (L), and (T); n = 45 (control) and n = 30 (mutant) cilia from three and two different lines, respectively (H, P, and X); ^∗p < 0.05; ^∗∗p < 0.01; ^∗∗∗∗p < 0.0001. Scale bar, 2.5 μm. See also Figures S6 and S7.