Transgenic animals may help resolve a sticky situation in cystic fibrosis

Depletion of Airway Submucosal Glands and TP63+KRT5+ Basal Cells in Obliterative Bronchiolitis

“…Although K5 1 p63 1 basal cells are less abundant in the distal airways of mice (12), our data are consistent with other studies in humans (16) and demonstrate that ferrets and humans share a highly similar distribution of basal cells in large and small airways. In addition, like humans, ferrets possess abundant SMGs throughout the cartilaginous airways (16,28,36,37), whereas mice possess SMGs only in the proximal trachea (36,38). These similarities in SC biology may account for the similarity between ferret and human OB development after lung transplantation.…”

Pancreatic pathophysiology in cystic fibrosis

“…In recent years, new CFTR ‐deficient animal models have been developed, including pig , ferret , rat and zebrafish . Animal modelling of CF in large animal species has advantages because these animals more closely resemble humans in terms of their lung structure, function and size . Of course, small animal models also have distinct advantages, which include ease of handling and housing as well as availability of reagents and tools for genetic manipulation .…”

Ferret and Pig Models of Cystic Fibrosis: Prospects and Promise for Gene Therapy

“…75 From an ion channel perspective, alternative non-CFTR, cAMP-activated, chloride channel activity appears to compensate for the lack of CFTR in the trachea 39,76 and pancreas in CF mice. [76][77][78] In contrast to mice, ferrets and pigs share a high level of similarity in airway cytoarchitecture with humans [79][80][81][82] and have a similar composition of chloride channels in the airway. 83 The use of the ferret as an animal model for hypersecretory diseases such as CF and chronic bronchitis was first suggested in 1982, based on the properties of mucus, goblet cells, and submucosal glands throughout the tracheobronchial tree.…”