How many functioning ciliated airway epithelial cells are necessary for effective mucociliary clearance?

Extract

Primary ciliary dyskinesia (PCD), the second most common genetic muco-obstructive lung disease, is caused by mutations in over 50 different genes that encode proteins that are critical for the structure and/or function of motile cilia expressed at the apical membrane of ciliated airway epithelial cells [1–5]. These PCD genes include genes encoding proteins of the axonemal structure, regulatory complexes, ciliary assembly or ciliary transport [2, 6], and their absence or dysfunction causes an inborn defect of mucociliary clearance of inhaled pathogens and irritants leading to impaired host defence of the lung [3, 4, 7].