Repurposing an old drug to treat cystic fibrosis airway disease

The lungs contain a thin layer of fluid known as the airway surface liquid (ASL), which helps protect against pathogens. The appropriate ASL volume, pH, and ionic composition are required for optimal airway defense. Cystic fibrosis (CF) is caused by expression of a dysfunctional cystic fibrosis transmembrane conductance regulator (CFTR), which acidifies the ASL and renders CF patients more susceptible to lung infections. In this issue of JCI Insight, Joseph Zabner and colleagues at the University of Iowa Carver College of Medicine examined the effect of tromethamine, a drug that is currently approved to treat metabolic acidosis, on ASL pH and bacterial killing activity. They demonstrated that inhalation of aerosolized tromethamine raised ASL pH in both pigs and CF patients. Importantly, tromethamine enhanced bacterial killing in the airways of pigs with CF and in sputum samples from humams with CF. These findings suggest that tromethamine may be beneficial in CF patients.

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TITLE:

Repurposing tromethamine as inhaled therapy to treat CF airway disease

AUTHOR CONTACT:

Joseph Zabner
University of Iowa Carver College of Medicine
Email: joseph-zabner@uiowa.edu

View this article at: http://insight.jci.org/articles/view/87535?key=7b80083f8876f1b1d56f

JCI Insight is the newest publication from the American Society of Clinical Investigation, a nonprofit honor organization of physician-scientists. JCI Insight is dedicated to publishing a range of translational biomedical research with an emphasis on rigorous experimental methods and data reporting. All articles published in JCI Insight are freely available at the time of publication. For more information about JCI Insight and all of the latest articles go to http://www.insight.jci.org.