Transgenic animals may help resolve a sticky situation in cystic fibrosis.
Cystic fibrosis (CF) is caused by defects in the CFTR, a cAMP-activated Cl- channel of epithelia. The resulting reduction in epithelial fluid transport creates abnormally viscous secretions from airway mucous glands that may be a major factor in CF pathology. Mouse airways have few mucous glands, and the mouse model of CF exhibits no significant airway disease. Pigs and ferrets, however, have approximately the same number of airway mucous glands as humans. In this issue of the JCI, three independent research groups conclude that changes in airway mucous gland function in CFTR-deficient animals of these species resemble the changes seen in human CF. It is expected, therefore, that these animals will develop lung disease similar to human CF and prove to be valuable models on which to test potential therapies.
Widdicombe
Jonathan H
JH
Department of Physiology and Membrane Biology, University of California Davis, Davis, California 95616-8664, USA. jhwiddicombe@ucdavis.edu
D000818:Animals; D030801:Animals, Genetically Modified; D001692:Biological Transport; D003550:Cystic Fibrosis / Q000473:pathology / Q000503:physiopathology* / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism; D004195:Disease Models, Animal; D004848:Epithelium / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D005088:Exocrine Glands / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D005289:Ferrets / Q000235:genetics*; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D013552:Swine / Q000235:genetics*
D019005:Cystic Fibrosis Transmembrane Conductance Regulator