Figure 5.

Modeling PCD-associated ciliary defects using AOAO rotation

(A) Diagram showing organoids engineered from hABSCs derived from PCD and healthy patients.

(B) Imaging of day 21 AOAOs (PCD and healthy) stained for Ac-α-Tub and ZO-1. Scale bar, 25 μm.

(C) Quantification of percentage ciliation in PCD and healthy AOAOs.

(D) TEM images of healthy and PCD AOAOs showing cilia ultrastructural defects in the PCD organoids (arrowheads). Scale bar, 100 nm.

(E) Diagram showing PCD and healthy AOAO’s rotational motion.

(F) Computational methods used for calculating angular velocities of healthy and PCD AOAOs.

(G) Rotation analysis of Matrigel-embedded, healthy, and PCD AOAOs.

(H) Kymograph-based CBF quantification of healthy and PCD AOAOs. ∗∗∗p < 0.001, Unpaired t-test.