This workshop will introduce two rodent models of CF (Î”F508 CFTR rat & a CF mouse expressing human CFTR) & provide new insights into biliary & respiratory CF disease from experiments performed using CF mouse, ferret or pig models. Particular emphasis will be given to evidence demonstrating infection- & inflammation-independent defects in CF airway function.
Summarize the advantages/disadvantages of new CF rodent models (the Î”F508 CFTR rat & human CFTR expressing mouse).
Explain implications of defective biliary ATP release in CF mice & age-dependent defects in CF ferret alveolar macrophages.
Summarize implications of sterile bronchiectasis in CF ferret lungs & inflammation-independent smooth muscle dysfunction in CF pig airways.