[Ion movement disorders in cystic fibrosis].
The most important of ion transport abnormalities is a reduced permeability to chlorine of certain epithelial cells, notably those of the sweat glands and bronchi. This abnormality is due to a defective regulation of the chlorine channels located in the apical membrane of these cells, and more precisely to the lack of activation of these channels by protein kinases A and C. Other epithelial ion transports are affected, but the mechanism of these disturbances is imperfectly known. In the airways, for instance, a decrease of chlorine secretion and an increase of sodium absorption concur to dehydrate the bronchial secretions and thus produce the respiratory manifestations of cystic fibrosis.
University of North Carolina, School of Medicine, Division of Pulmonary Diseases, Chapel Hill 27599-7020.
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D001692:Biological Transport; D003550:Cystic Fibrosis / Q000378:metabolism*; D004848:Epithelium / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007473:Ion Channels / Q000378:metabolism*; D012137:Respiratory System / Q000378:metabolism; D014883:Water-Electrolyte Imbalance / Q000378:metabolism*
D007473:Ion Channels
Les troubles des mouvements ioniques dans la mucoviscidose.