Airway disease currently causes most of the morbidity and mortality in patients with cystic
fibrosis (CF). However, understanding the pathogenesis of CF lung disease and developing …

Animal models of human diseases are critical for dissecting mechanisms of pathophysiology
and developing therapies. In the context of cystic fibrosis (CF), mouse models have been the …

Lung disease causes most of the morbidity and mortality in cystic fibrosis (CF).
Understanding the pathogenesis of this disease has been hindered, however, by the lack of …

In humans, cystic fibrosis (CF) lung disease is characterised by chronic infection,
inflammation, airway remodelling, and mucus obstruction. A lack of pulmonary …

Cystic fibrosis (CF) results from mutations that disrupt CF transmembrane conductance
regulator (CFTR), an anion channel found mainly in apical membranes of epithelial cells. CF …

Basic and clinical cystic fibrosis research will benefit greatly from the comparative
pathophysiology of cystic fibrosis mice, pigs, and ferrets. Both similarities and differences …

Cystic Fibrosis (CF) is a common autosomal recessive disease that affects multiple organs.
The lack of an animal model with manifestations like those typically found in humans has …

Animal models of cystic fibrosis (CF) are powerful tools that enable the study of the
mechanisms and complexities of human disease. Murine models have several intrinsic …

The leading cause of mortality and morbidity in humans with cystic fibrosis is lung disease.
Advances in our understanding of the pathogenesis of the lung disease of cystic fibrosis, as …

Rationale: Although airway abnormalities are common in patients with cystic fibrosis (CF), it
is unknown whether they are all secondary to postnatal infection and inflammation, which …