[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.
Chinet
T
T
University of North Carolina, School of Medicine, Division of Pulmonary Diseases, Chapel Hill 27599-7020.
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.