New research by University of Iowa scientists shows that cystic fibrosis (CF), a life-shortening, inherited condition that affects about 30,000 Americans, causes a specific defect in this process, reducing the ability to clear particles and germs out of the airway.
In CF, mucus abnormalities are linked to serious health problems, including airway obstruction, difficulty breathing, and increased susceptibility to lung infection. However, whether the mucus abnormality is a primary defect in CF, or is a secondary consequence of CF lung disease, has been an unanswered question.
Using a pig model of CF, which more closely mimics human disease than the traditional mouse experimental models, the UI team found that the mucus defect is present at birth and is a primary cause of CF lung disease. The results are published in the Aug. 15 issue of the journalScience.