The porcine lung as a potential model for cystic fibrosis.
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.
Rogers
Christopher S
CS
Department of Internal Medicine, Roy J. Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242, USA.
Abraham
William M
WM
Brogden
Kim A
KA
Engelhardt
John F
JF
Fisher
John T
JT
McCray
Paul B
PB
McLennan
Geoffrey
G
Meyerholz
David K
DK
Namati
Eman
E
Ostedgaard
Lynda S
LS
Prather
Randall S
RS
Sabater
Juan R
JR
Stoltz
David Anthony
DA
Zabner
Joseph
J
Welsh
Michael J
MJ
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*
D019005:Cystic Fibrosis Transmembrane Conductance Regulator