Abstract:
: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.
journal_name
Nat Genetjournal_title
Nature geneticsauthors
Davidson DJ,Dorin JR,McLachlan G,Ranaldi V,Lamb D,Doherty C,Govan J,Porteous DJdoi
10.1038/ng0495-351subject
Has Abstractpub_date
1995-04-01 00:00:00pages
351-7issue
4eissn
1061-4036issn
1546-1718journal_volume
9pub_type
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