Mouse Genome Informatics
hm
    Cftrtm1Hgu/Cftrtm1Hgu
involves: 129P2/OlaHsd
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
immune system
• mutants exposed to S. aureus exhibit increased lymphoid infiltrate and bronchiolitis compared to wild-type
• mutants exposed to B. cepacia exhibit much more severe bronchopneumonia and mixed inflammatory infiltrate than wild-type
• mutants are unable to clear Staphylococcus aureus and Burkholderia cepacia from the lungs and develop lung disease after repeated bacterial exposure

respiratory system
N
• under normal conditions (without bacterial exposure), no lung abnormalities are detected in homozygotes (J:103284)
• mutants exposed to S. aureus exhibit increased lymphoid infiltrate and bronchiolitis compared to wild-type
• mutants exposed to B. cepacia exhibit much more severe bronchopneumonia and mixed inflammatory infiltrate than wild-type
• mutants exhibit lung disease in response to repeated bacterial exposure
• mutants exhibit increased lymphoid infiltrate, goblet cell hyperplasia, mucus retention in the airways, bronchiolitis compared to wild-type after S. aureus infection
• mutants exhibit severe bronchopneumonia and mucus retention in the major airways, goblet cell hyperplasia and metaplasia, massive mixed inflammatory infiltrate, obliteration of smaller airways, and extensive oedema compared to wild-type after B. cepacia infection
• mutants exposed to bacteria (S. aureus and B. cepacia) exhibit increased goblet cell hyperplasia compared to wild-type

Mouse Models of Human Disease
OMIM IDRef(s)
Cystic Fibrosis; CF 219700 J:24119