Females with cystic fibrosis have more severe disease than males with cystic fibrosis and have a shorter lifespan. Although many suggestions have been put forward to explain this sex-related difference, a concrete mechanism to explain it has remained elusive.
However, Robert Tarran and colleagues, at University of North Carolina, Chapel Hill, have now uncovered a molecular mechanism whereby the hormone estrogen has a negative impact on the clearance of mucus from the lungs; the buildup of mucus in the lung is a hallmark of cystic fibrosis and serves as a focus for severe infections.
As the estrogen antagonist tamoxifen blocked one aspect of the molecular mechanism in vitro, the authors suggest that antiestrogens might be beneficial in the treatment of cystic fibrosis.
In an accompanying commentary, Pamela Zeitlin, at Johns Hopkins School of Medicine, Baltimore, outlines the complexities of the molecular mechanism uncovered by Tarran and colleagues, and further discusses the clinical implications of the data.
- Coakley et al. 17β-Estradiol inhibits Ca2+-dependent homeostasis of airway surface liquid volume in human cystic fibrosis airway epithelia. Journal of Clinical Investigation, 2008; DOI: 10.1172/JCI33893
- Zeitlin. Cystic fibrosis and estrogens: a perfect storm. Journal of Clinical Investigation, 2008; DOI: 10.1172/JCI37778
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