Article
Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis.
The Journal of clinical investigation - 1 Sept 2010
Sun Xingshen, Sui Hongshu, Fisher John T, Yan Ziying, Liu Xiaoming, Cho Hyung-Ju, Joo Nam Soo, Zhang Yulong, Zhou Weihong, Yi Yaling, Kinyon Joann M, Lei-Butters Diana C, Griffin Michelle A, Naumann Paul, Luo Meihui, Ascher Jill, Wang Kai, Frana Timothy, Wine Jeffrey J, Meyerholz David K, Engelhardt John F
Abstract excerpt
Cystic fibrosis (CF) is a recessive disease that affects multiple organs. It is caused by mutations in CFTR. Animal modeling of this disease has been challenging, with species- and strain-specific differences in organ biology and CFTR function influencing the emergence of disease pathology. Here, we report the phenotype of a CFTR-knockout ferret model of CF. Neonatal CFTR-knockout ferrets demonstrated many of the...
Topics
- Animals
- Animals, Newborn
- Cells, Cultured
- Cystic Fibrosis
- Cystic Fibrosis Transmembrane Conductance Regulator
- Disease Models, Animal
- Ferrets
- Humans
- Lung