FIGURE 3.
Persistent ultrastructural defects in primary ciliary dyskinesia (PCD) cell cultures. Electron micrographs of representative ciliary cross-sections illustrating examples of the defects observed in both initial patient brushings (upper panels) and in air–liquid interface (ALI) cultures derived from 3T3+Y PCD primary epithelial cell cultures. For each patient, 3T3+Y-derived ALI cultures were assessed as follows: PCD 1 (219 cilia; 97.7% IDA, 2.3% no DA), PCD 2 (305 cilia; 100% DA), PCD 6 (231 cilia; 97.4% ODA, 2.6% no DA), PCD 11 (269 cilia; 98.1% ODA, 1.9% no DA; 13.6% microtubular defect) and PCD 12 (265 cilia; 98.1% normal, 0.4% ODA, 1.5% no DA; 9.3% transposed). ODA: outer dynein arm; IDA: inner dynein arm; DA: dynein arm. #: radial spoke refers to axonemal disorganisation with absent IDAs.