Scientists at the University of Iowa have made a significant discovery that sheds new light on the underlying cause of cystic fibrosis (CF), a genetic disease primarily affecting the lungs and other organs. CF is characterized by the absence or mutation of a protein called the cystic fibrosis transmembrane conductance regulator (CFTR) in the lungs.
Traditionally, research on CFTR proteins has focused on airway secretory cells, where CFTR channels help regulate the balance of salt and water on the lung’s surface. In these cells, chloride ions are secreted into the thin layer of liquid covering the airway surface, which helps maintain the right consistency for mucus to trap and expel irritants and pathogens. However, individuals with CF have thick and sticky mucus, which can block airways.
In 2018, researchers discovered a new type of cell in the human lung called ionocytes, similar to those found in fish and frogs. These cells make up only 1% of all lung cells but contain most of the CFTR channels in the human body. A recent study at the University of Iowa cultured ionocytes in the lab and found that CFTR proteins behave differently in these cells.