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Nature Medicine 9, 827 - 828 (2003)
doi:10.1038/nm0703-827
Rules of conduct for the cystic fibrosis anion channel
Jeffrey J Wine1
- The author is in the Cystic Fibrosis Research Lab, Department of Psychology, Stanford University, Stanford, California 94305-2130, USA. e-mail: wine@psych.stanford.edu
Abstract
Improper ion balance resulting from defects in the ion channel CFTR underlies cystic fibrosis. Separate control mechanisms are now shown to regulate the flux of chloride and bicarbonate through this channel.
Investigations into cystic fibrosis transmembrane conductance receptor (CFTR), the ion channel defective in cystic fibrosis, have yielded a triple surprise. CFTR, which regulates anion flux in wet epithelial tissues that line organs such as the airways, intestines and sweat glands, was once thought to be a relatively nonselective anion channel activated by kinase-mediated phosphorylation and binding of ATP.
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