Cure for cystic fibrosis may be found in airway cell type
In separate studies published online today in Nature, two independent research teams report the discovery of a new, rare type of cell in the human airway. These cells appear to be the primary source of activity of the CFTR gene, mutations which cause cystic fibrosis, a multiorgan disease that affects more than 70,000 people worldwide. The CFTR gene provides instructions for making a protein called the cystic fibrosis transmembrane conductance regulator. This protein functions as a channel across the membrane of cells that produce mucus, sweat, saliva, tears, and digestive enzymes. Despite decades of study on CFTR and progress in treating cystic fibrosis, there is still no cure. The new findings reveal that CFTR activity is concentrated in a small, previously unknown population of cells, which may serve as promising targets for future therapeutic strategies against the disease. The researchers named the cells “pulmonary ionocytes” due to similarities with ionocytes, a type of cell that regulates salt balance and is found in the...