Cilia dysfunction in lung disease.

Abstract:

: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.

journal_name

Annu Rev Physiol

authors

Tilley AE,Walters MS,Shaykhiev R,Crystal RG

doi

10.1146/annurev-physiol-021014-071931

subject

Has Abstract

pub_date

2015-01-01 00:00:00

pages

379-406

eissn

0066-4278

issn

1545-1585

journal_volume

77

pub_type

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