Journal
ANNUAL REVIEW OF PHYSIOLOGY, VOL 77
Volume 77, Issue -, Pages 379-+Publisher
ANNUAL REVIEWS
DOI: 10.1146/annurev-physiol-021014-071931
Keywords
airway; epithelium; cilia; mucociliary escalator
Categories
Funding
- NHLBI NIH HHS [R01 HL123544, U01HL121828, R01HL118857, R01HL107882, R01 HL118857, R01 HL107882, K23 HL103837, U01 HL121828] Funding Source: Medline
- NATIONAL HEART, LUNG, AND BLOOD INSTITUTE [R01HL107882, R01HL118857, K23HL103837, U01HL121828] Funding Source: NIH RePORTER
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A characteristic feature of the human airway epithelium is the presence of ciliated cells bearing motile cilia, specialized cell surface projections containing axonemes composed of microtubules and dynein arms, which provide ATP-driven motility. In the airways, cilia function in concert with airway mucus to mediate the critical function of mucociliary clearance, cleansing the airways of inhaled particles and pathogens. The prototypical disorder of respiratory cilia is primary ciliary dyskinesia, an inherited disorder that leads to impaired mucociliary clearance, to repeated chest infections, and to the progressive destruction of lung architecture. Numerous acquired lung diseases are also marked by abnormalities in both cilia structure and function. In this review we summarize current knowledge regarding airway ciliated cells and cilia, how they function to maintain a healthy epithelium, and how disorders of cilia structure and function contribute to inherited and acquired lung disease.
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