Mucus Retained in Goblet Cells of Cystic Fibrosis Patients Leads to Potentially Deadly Infections; New Finding Could Lead to Improved Treatments

Cystic fibrosis (CF) is a genetic disorder that affects one out of every 3,000 children in populations of Northern European descent. One of the key signs of CF is that mucus lining the lungs, pancreas, and other organs is too sticky, which makes it difficult for the organs to work properly, and the CF mucus in the lungs attracts bacteria and viruses resulting in chronic infections. Researchers at the University of Missouri-Columbia (MU-Columbia) recently found that CF mucus actually gets stuck inside some of the cells (goblet cells) that create it, rather than simply becoming stuck on the outside linings of organs. Dr. Lane Clarke, a Professor of Biomedical Sciences in the MU College of Veterinary Medicine, says that now that it is better understood how mucus becomes trapped in the body, scientists can begin working on potential treatments for patients with CF that help cells remove the sticky mucus more quickly. "Normally, special cells (goblet cells) create mucus and easily push it out to the linings of the organs where it belongs," said Dr. Clarke, who also is a Dalton Investigator in the MU Dalton Cardiovascular Research Center. "However, in CF patients, some cells that create the mucus fail to completely release the mucus, so the mucus becomes stuck halfway in and halfway out. This makes mucus clearance more difficult and potentially would allow bacteria to have an easy pathway to infecting cells to cause diseases like pneumonia." Dr. Clarke also examined the characteristics of mucus stored within the cells and found that it is not as acidic as in normal cells. "Previously, CF researchers disagreed as to whether CF cells also have a defect in properly acidifying areas inside cells," Dr. Clarke said.
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