UNC Health Talk

Increased mucins pinned to worsening cystic fibrosis symptoms

Media Contact: Mark Derewicz, 919-923-0959, mark.derewicz@unchealth.unc.edu

June 2, 2014

CHAPEL HILL, N.C. – UNC School of Medicine researchers have provided the first quantitative evidence that mucins – the protein framework of mucus – are significantly increased in cystic fibrosis patients and play a major role in failing lung function.

The research, published today in the Journal of Clinical Investigation, shows that a three-fold increase of mucins dramatically increases the water-draining power of the mucus layer. This hinders mucus clearance in the CF lung, resulting in infection, inflammation, and ultimately lung failure.

“Our finding suggests that diluting the concentration of mucins in CF mucus is a key to better treatments,” said Mehmet Kesimer, PhD, associate professor of pathology and laboratory medicine and co-senior author of the JCI paper.

Ashley Henderson, MD, assistant professor of medicine and co-first author of the JCI paper, added, “We think this study shows why nebulized hypertonic saline [sterile salty water] improves the hydration of the CF airway, improves the patient’s mucus clearance and, in so doing, increases lung function.”

The UNC study also casts further doubt on a controversial 2004 study that disputed the theory that mucins play a major role in CF.

This work, a collaboration of 13 UNC scientists, is part of an extensive UNC lung research program based in the new Marsico Lung Institute, which is led by Richard Boucher, MD, co-senior author of the JCI paper.