![]() |
Hydration restores the airway surface seal. In turquoise, the junctions between the epithelial cells (blue). Credit: UNIGE - Laboratory of Prof. Marc Chanson |
Cystic fibrosis is a rare genetic disease which can cause very serious symptoms. In particular, patients suffer from chronic bacterial infections that can lead to respiratory failure. It is caused by mutations in the CFTR gene, which regulates water movement across the cell membrane. Consequently, mucus quality is altered, it is no longer capable of capturing undesirable bacteria and expelling them. Using a model reproducing a respiratory epithelium - a protective tissue composed of a monolayer of cells - teams from the University of Geneva (UNIGE) have discovered that a simple film of liquid is sufficient to restore the airways’ seal and reduce the risk of bacterial infection. These results, to be read in a special issue of the journal Cells, open the way to new therapies based on mucus hydration. A promising alternative to current therapies that are often not widely enough effective.
Despite recent therapeutic advances, people with cystic fibrosis — one in every 2,500 births in Europe — have a life expectancy of no more than 46 years and altered quality of life. The disease is caused by one or more mutations in the CFTR gene, which affects the proper functioning of an essential protective barrier. The epithelial cells that line the airways are usually sealed together and thus protect the airways from bacterial colonization. They are also lined with a fluid, a slippery mucus that traps unwanted germs and carries them away. When the CFTR protein is altered, the junctions between the cells loosen and the dehydrated mucus tends to stagnate, both of which promote the development of respiratory