Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences

Table 2

Phenotypic manifestations of cystic fibrosis in humans and animal models.

	Spontaneous lung infection	Pancreatic disease	Intestinal disease	Liver and gallbladder disease	Reproduction
Human	Yes	PI	MI	Biliary cirrhosis	Severe vas deferens defect
Mouse	No	No	Intestinal obstruction, often fatal	No	Reduced fertility in females
Pig	Yes	PI	100% MI	Biliary cirrhosis	Severe vas deferens defect
Ferret	Yes	PI	75% MI	Liver disease	Severe vas deferens defect

The phenotypic manifestations outlined above may differ between mouse models. PI: pancreatic insufficiency; MI: meconium ileus.