The human airway is a pretty inhospitable place for microbes. There are numerous immune defense mechanisms poised to kill or remove inhaled bacteria before they can cause problems. But cystic fibrosis (CF) disrupts these defenses, leaving patients particularly susceptible to airway infection, which is the major cause of disease and death in CF. Using a unique animal model of CF, a team of scientists from the University of Iowa has discovered a surprising difference between healthy airways and airways affected by CF that leads to reduced bacterial killing in CF airways. The finding directly links the genetic cause of CF -- mutations in a channel protein called cystic fibrosis transmembrane conductance regulator (CFTR) -- to the disruption of a biological mechanism that protects lungs from bacterial infection.
