The use of animal models in pathology is a necessity without which studying human genetic disorders and developing therapies wouldn’t be possible. In the past, cystic fibrosis has been studied on human patients and mouse models. Despite effectiveness of mouse models that allowed to gather invaluable information about the disease, they however don’t fully recapitulate the natural progression of cystic fibrosis observed in humans. Because of that, the scientists developed two additional cystic fibrosis models – in the pig and ferret. This helped to observe many phenotypic similarities to the human disease. Besides that, refinements made in the cystic fibrosis mouse models using CFTR (organ-specific conditional cystic fibrosis transmembrane conductance regulator) started to inform new functions of CFTR in tissues that previously were overlooked (1).
The benefit of using the cell-specific transgenic complementation approaches in cystic fibrosis pigs and ferrets models is that not only it generates animal models which are easier to rear, but it also proves useful in dissecting the contribution of CFTR in various tissues to disease progression in the whole animal. Also, the spontaneous lung and pancreatic phenotypes observed in the cystic fibrosis pig and ferret models allow scientists to study disease progression in more details as these organs are less approachable in mice (1).
