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University of Iowa: Cystic fibrosis pig study shows airways are more acidic, reduces bacterial killing

Using a unique animal model of cystic fibrosis, a team of scientists from the University of Iowa has discovered a difference between healthy airways and airways affected by cystic fibrosis that leads to reduced bacterial killing in cystic fibrosis airways.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 disrupts these defenses, leaving patients particularly susceptible to airway infection, which is the major cause of disease and death in cystic fibrosis.

Using a unique animal model of cystic fibrosis, a team of scientists from the University of Iowa has discovered a difference between healthy airways and airways affected by cystic fibrosis that leads to reduced bacterial killing in cystic fibrosis airways. The finding directly links the genetic cause of cystic fibrosis—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.