Mouse models of cystic fibrosis.
The development of mouse models for cystic fibrosis has provided the opportunity to dissect disease pathogenesis, correlate genotype and phenotype, study disease-modifying genes and develop novel therapeutics. This review discusses the successes and the challenges encountered in characterizing and optimizing these models.
Davidson
D J
DJ
University of British Columbia, BC Research Institute for Child and Family Health, Room 381, 950 West 28th Avenue, British Columbia, V5Z 4H4., Vancouver, Canada. donald@cmdr.ubc.ca
Rolfe
M
M
D000818:Animals; D003550:Cystic Fibrosis / Q000209:etiology / Q000235:genetics* / Q000628:therapy; D004195:Disease Models, Animal; D006801:Humans; D007410:Intestinal Diseases / Q000503:physiopathology; D008171:Lung Diseases / Q000473:pathology / Q000503:physiopathology; D051379:Mice; D008817:Mice, Mutant Strains; D010182:Pancreatic Diseases / Q000503:physiopathology; D010641:Phenotype