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DOI: 10.1080/01926230601060025 © 2007 Society of Toxicologic Pathology
Cystic Fibrosis and Other Respiratory Diseases of Impaired Mucus Clearance
1 Cystic Fibrosis/Pulmonary Research and Treatment Center, Department of Medicine, The University of North Carolina at Chapel Hill Correspondence: Address correspondence to: Scott H. Randell, UNC CF Center, CB 7248, Room 4011 Thurston-Bowles Building, Chapel Hill, NC 27599; e-mail:randell{at}med.unc.edu Exposed to a diverse array of potentially noxious agents, the respiratory tract is protected by a highly developed innate defense system. Physiologically regulated epithelial ion and water transport coordinated with mucin secretion, beating cilia, and cough results in continuous flow of fluid and mucus over airway surfaces toward the larynx. This cleansing action is the initial and perhaps most quantitatively important innate defense mechanism. Repeated lung infections and eventual respiratory insufficiency characteristic of human cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) illustrate the consequences of impaired mucus clearance. Altered mucus clearance likely contributes to the initiation, progression, and chronicity of other airway diseases characterized by inflammation and mucous secretory cell hyper/metaplasia that afflict millions worldwide, including chronic obstructive pulmonary disease (COPD). This review concisely discusses the pathophysiology of human diseases characterized by genetic defects that impair mucus clearance. It then explores animal models in which components of the mucus clearance system have been disrupted. These models firmly establish the importance of mucus clearance for respiratory health, and will help elucidate disease mechanisms and therapeutic strategies in CF, PCD and COPD.
Key Words: Chronic obstructive pulmonary disease • cilia • mucin • mucociliary clearance • cystic fibrosis • primary ciliary dyskinesia Abbreviations: CF, cystic fibrosis • PCD, primary ciliary dyskinesia • COPD, chronic obstructive pulmonary disease • PCL, peri-ciliary layer • ASL, airway surface liquid • CFTR, cystic fibrosis transmembrane conductance regulator • ENaC, epithelial sodium channel • OMIM, Online Mendelian Inheritance in Man • TEM, transmission electron microscopy • PD, potential difference • Isc, short circuit current
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