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“Introduction The average human inhales ~10,000 L of air every day. Respiration is a portal of entry for not only atmospheric gases, but also for harmful particulate pervasive in the environment. The pulmonary epithelium is therefore continually exposed to microorganisms, but remains sterile under normal physiologic conditions. This remarkable phenomenon is a testament to the innate immune defenses that provide a silent mode of broad immune protection. The importance of the innate immune system in protecting the lungs

from infection is clearly illustrated in the pathologic condition that arises in cystic fibrosis (CF) (mucoviscidosis), which severely damages the pulmonary innate immune defenses [1]. Cystic fibrosis is the most common lethal genetic disorder affecting the Caucasian population, Org 27569 with an incidence of 1 in 2,500 births [2]. CF is caused by an autosomal recessive mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene within chromosome seven [3]. This mutation results in the functional defect in the cyclic adenosine monophosphate stimulated pulmonary chloride pump causing abnormal ion transport in epithelial cells [4, 5]. CF is therefore a disease of ion transport across the epithelium, affecting fluid secretion in exocrine JNJ-26481585 purchase glands and the epithelium of the respiratory, reproductive, and gastrointestinal tracts [6]. Although CF causes a multitude of pathophysiologic effects, the most significant effect is the impaired ciliary clearance that results in the accumulation of mucus in the lung, creating a haven for bacteria.

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