Cilia dysfunction in lung disease

Ann E. Tilley, Matthew S. Walters, Renat Shaykhiev, Ronald Crystal

Research output: Contribution to journalArticle

84 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)379-406
Number of pages28
JournalAnnual Review of Physiology
Volume77
DOIs
Publication statusPublished - 1 Jan 2015
Externally publishedYes

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Keywords

  • Airway
  • Cilia
  • Epithelium
  • Mucociliary escalator

ASJC Scopus subject areas

  • Physiology

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