Altered NaCl concentration of airway surface liquid in cystic fibrosis.
A novel isotopic technique suggests that the [Na] and [Cl] of airway surface liquid are both normally approximately 50 mM. In cystic fibrosis, lack of the functional cystic fibrosis transmembrane conductance regulator (CFTR) causes failure of transcellular Cl absorption, resulting in an elevation of [Na] and [Cl] of airway surface liquid to approximately 100 mM.
Widdicombe
J H
JH
Department of Human Physiology, University of California - Davis, Davis, CA 95616-8664, USA. jhwiddicombe@ucdavis.edu
D002478:Cells, Cultured; D003550:Cystic Fibrosis / Q000378:metabolism*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism; D006801:Humans; D009995:Osmosis; D020545:Respiratory Mucosa / Q000166:cytology / Q000378:metabolism*; D012965:Sodium Chloride / Q000378:metabolism*; D013499:Surface Properties; D014867:Water / Q000378:metabolism
C505032:CFTR protein, human; D014867:Water; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D012965:Sodium Chloride