A research group from the University of Iowa (USA) generated pigs in which both CFTR alleles were disrupted. Two decades ago CFTR was identified as the gene causing cystic fibrosis, however a cure to the disease has not been established yet.

In mouse models, the characteristic manifestations of human cystic fibrosis-like abnormalities of the pancreas, lung, intestine, liver could not be generated. Since pigs share many anatomical and physiological features with humans, they were chosen as model to target the CFTR gene.

Piglets without functional CFTR showed defective chloride transport and developed meconium ileus, exocrine pancreatic destruction, and focal biliary cirrhosis. Those abnormalities have also been observed in newborn humans with cystic fibrosis. The pig model may hence hold answers on the ultimate questions of cystic fibrosis pathogenesis and speed up the process of discovering strategies for prevention and treatment.

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