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Lung disease is the major cause of death in cystic fibrosis (CF), but there is no evidence for overt lung involvement at birth. We show here that the same is true for the gene targeted cftrm1HGU mutant mouse. Furthermore, this CF mouse model demonstrates an impaired capacity to clear Staphylococcus aureus and Burkholderia (Pseudomonas) cepacia, two opportunistic lung pathogens closely associated with lung disease in CF subjects. The cftrm1HGU homozygotes display mucus retention and frank lung disease in response to repeated microbial exposure. Thus, lung disease in the cftrm1HGU mouse develops in response to bacterial infection, establishing a model to dissect the pathogenesis of CF pulmonary disease and providing a clinically relevant end point to assess the efficacy of pharmacologic or genetic interventions.
D J Davidson, and
J R Dorin, and
G McLachlan, and
V Ranaldi, and
D Lamb, and
C Doherty, and
J Govan, and
D J Porteous
January 2011,
Methods in molecular biology (Clifton, N.J.),
D J Davidson, and
J R Dorin, and
G McLachlan, and
V Ranaldi, and
D Lamb, and
C Doherty, and
J Govan, and
D J Porteous
July 2014,
The international journal of biochemistry & cell biology,
D J Davidson, and
J R Dorin, and
G McLachlan, and
V Ranaldi, and
D Lamb, and
C Doherty, and
J Govan, and
D J Porteous
February 2016,
Medecine et maladies infectieuses,
D J Davidson, and
J R Dorin, and
G McLachlan, and
V Ranaldi, and
D Lamb, and
C Doherty, and
J Govan, and
D J Porteous
August 2008,
The European respiratory journal,
D J Davidson, and
J R Dorin, and
G McLachlan, and
V Ranaldi, and
D Lamb, and
C Doherty, and
J Govan, and
D J Porteous
May 2015,
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society,
D J Davidson, and
J R Dorin, and
G McLachlan, and
V Ranaldi, and
D Lamb, and
C Doherty, and
J Govan, and
D J Porteous
September 2022,
Journal of the Pediatric Infectious Diseases Society,
D J Davidson, and
J R Dorin, and
G McLachlan, and
V Ranaldi, and
D Lamb, and
C Doherty, and
J Govan, and
D J Porteous
June 2005,
Respiratory research,
D J Davidson, and
J R Dorin, and
G McLachlan, and
V Ranaldi, and
D Lamb, and
C Doherty, and
J Govan, and
D J Porteous
April 1983,
Lancet (London, England),
D J Davidson, and
J R Dorin, and
G McLachlan, and
V Ranaldi, and
D Lamb, and
C Doherty, and
J Govan, and
D J Porteous
February 2017,
Pediatric pulmonology,
D J Davidson, and
J R Dorin, and
G McLachlan, and
V Ranaldi, and
D Lamb, and
C Doherty, and
J Govan, and
D J Porteous
September 2021,
Microbiology spectrum,
