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.