Lessons from the CFTR knockout swine: reduced airway surface liquid secretion and Na+ hyperabsorption contribute to lung disease.
Using swine models of cystic fibrosis (CFTR knockout pigs) we studied the pathophysiology of Airway Surface Liquid (ASL) secretion. We developed a synchrotron-based Phase Contrast Imaging (PCI) method and a high-resolution self-referencing Na+-selective microelectrode technique to study the mechanism of ASL secretion and how it malfunctions in CF. Our results suggest that CF airways would fail to appropriately respond to inhaled bacteria, resulting in reduced secretion of mucin and antimicrobial factors required to clear bacteria, thus facilitating infection. Moreover, CFTR-/- swine airway suffers from ENaC-dependent Na+ reabsorption that likely contributes to CF pathogenesis in the distal airways.
- Conférencier/Speaker: Dr. Juan Ianowski
- Institution(s), Partenaire(s) / Institution(s), Sponsor(s): Associate Professor, Department of Anatomy, Physiology and Pharmacology University of Saskatchewan