Abstract
Mutation of the CFTR chloride channel was identified as the genetic basis of cystic fibrosis over 20 years ago; however, correlation of the pathophysiological changes occurring in CF lung disease with the mutation of a chloride channel is ongoing. The failure of innate lung defense in CF, and the subsequent cyclical microbial colonization of airways, explains the gross anatomical changes that occur in CF pathophysiology. However, ongoing research is focused on how the lack of the CFTR channel explains the failure of innate lung defense. Hydration status of the mucus blanket is key to understanding this link, and this series of chapters details the recent progress that has been made in understanding the interplay between ion transport activity and innate lung defense, and the initiation of CF lung pathophysiology.
MeSH terms
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Airway Remodeling
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Animals
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Bacterial Infections / microbiology
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Bacterial Infections / physiopathology
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Biofilms
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Biological Transport
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Cystic Fibrosis / genetics
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Cystic Fibrosis / metabolism
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Cystic Fibrosis / microbiology
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Cystic Fibrosis / physiopathology*
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Cystic Fibrosis Transmembrane Conductance Regulator / genetics
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Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
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Dehydration / metabolism
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Dehydration / physiopathology
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Humans
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Inflammation / metabolism
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Inflammation / microbiology
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Inflammation / physiopathology
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Lung / metabolism
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Lung / microbiology
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Lung / physiopathology*
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Mice
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Mucociliary Clearance
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Mucus / metabolism*
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Mucus / microbiology
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Mutation
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Neutrophil Infiltration
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Osmotic Pressure
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Research Design
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Water / metabolism*
Substances
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Water
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Cystic Fibrosis Transmembrane Conductance Regulator