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Updated: Aug 29, 2026

Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein
Published on: March 9, 2015
Chloride channels, Golgi pH and cystic fibrosis
1Department of Medicine, College of Physicians and Surgeons of Columbia University, 630 W. 168th St, New York, NY 10032, USA.
Abstract:
Cystic fibrosis (CF) is associated with a defect in a cAMP-activated chloride channel in secretory epithelia, which leads to decreased fluid secretion. In addition, many mucus glycoproteins show decreased sialylation but increased sulphation. We have recently shown that the pH of intracellular organelles is elevated in CF cells, due to defective chloride conductance in the vesicle membranes. We postulate that this may affect the activity of sialyl-, fucosyl- and sulphotransferases, and thus explain the abnormal glycosylation. Defects in sialylation of glycolipids might also generate receptors for Pseudomonas, which infects the respiratory tract of CF patients.
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