title: The porcine lung as a potential model for cystic fibrosis.
abstract: Airway disease currently causes most of the morbidity and mortality in patients with cystic fibrosis (CF). However, understanding the pathogenesis of CF lung disease and developing novel therapeutic strategies have been hampered by the limitations of current models. Although the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) has been targeted in mice, CF mice fail to develop lung or pancreatic disease like that in humans. In many respects, the anatomy, biochemistry, physiology, size, and genetics of pigs resemble those of humans. Thus pigs with a targeted CFTR gene might provide a good model for CF. Here, we review aspects of porcine airways and lung that are relevant to CF.
authors.affiliation: Department of Internal Medicine, Roy J. Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242, USA.
authors.forename: Christopher S
authors.initials: CS
authors.lastname: Rogers
authors.forename: William M
authors.initials: WM
authors.lastname: Abraham
authors.forename: Kim A
authors.initials: KA
authors.lastname: Brogden
authors.forename: John F
authors.initials: JF
authors.lastname: Engelhardt
authors.forename: John T
authors.initials: JT
authors.lastname: Fisher
authors.forename: Paul B
authors.initials: PB
authors.lastname: McCray
authors.forename: Geoffrey
authors.initials: G
authors.lastname: McLennan
authors.forename: David K
authors.initials: DK
authors.lastname: Meyerholz
authors.forename: Eman
authors.initials: E
authors.lastname: Namati
authors.forename: Lynda S
authors.initials: LS
authors.lastname: Ostedgaard
authors.forename: Randall S
authors.initials: RS
authors.lastname: Prather
authors.forename: Juan R
authors.initials: JR
authors.lastname: Sabater
authors.forename: David Anthony
authors.initials: DA
authors.lastname: Stoltz
authors.forename: Joseph
authors.initials: J
authors.lastname: Zabner
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Welsh
mesh_terms: D000818:Animals; D003550:Cystic Fibrosis / Q000150:complications / Q000235:genetics* / Q000378:metabolism / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics* / Q000378:metabolism*; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D010182:Pancreatic Diseases / Q000150:complications / Q000235:genetics / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D013552:Swine / Q000235:genetics* / Q000378:metabolism*
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator