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J. Biol. Chem., Vol. 281, Issue 11, 99904, March 17, 2006

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Mucus Clearance in Cystic Fibrosis

Cystic fibrosis is a genetic disorder resulting from mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. One symptom of the disease is chronic bacterial infection of the airways and sinuses due to impaired ability to clear mucus from the airways. Currently, there are two essentially opposite explanations for this dysfunction. The first is that CFTR-mediated fluid secretion is decreased in cystic fibrosis airway glands. The second explanation is that the glands still secrete fluid via CFTR-independent pathways, and the exit of mucus from the airways results from hyperabsorption of fluid by the gland ducts. This could occur if CFTR loss uninhibited the epithelial Na⁺ channel (ENaC), which drives absorption.

In this Paper of the Week, Nam Soo Joo and colleagues provide convincing evidence that hyposecretion, not hyperabsorption, is the basic defect in cystic fibrosis airway glands. To test the hyperabsorption hypothesis, Joo et al. used optical methods to study secretion rates of submucosal glands from patients with cystic fibrosis and from normal pigs in the presence and absence of ENaC inhibitors. The researchers found no evidence that the inhibitors altered secretion rates in either normal or cystic fibrosis glands, consistent with the conclusion that loss of CFTR-mediated fluid secretion is the culprit in cystic fibrosis.

FOOTNOTES

See referenced article, J. Biol. Chem. 2006, 281, 7392-7398

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This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Search for Related Content Related Collections Papers Of The Week Social Bookmarking                      What's this?