Mouse Genome Informatics
hm
    Cftrtm1Cam/Cftrtm1Cam
involves: 129S/SvEv
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• pups fail to thrive and about 80% die 2-5 days after birth, the rest survive up to 45 days, with a large percentage of survivors dying around 21 days (J:23850)
• a few survive up to 45 days (J:23850)

digestive/alimentary system
• dilation and blockage of pancreatic ducts in 5 of 10 mutants aged 17 days or older
• distension of the intestine in observed proximally to the blockage and within the distended area, the majority of the intestinal villi are severely atrophic and necrotic in places
• crypts of Lieberkuhn are severely dilated with mucus
• cecum is reduced
• crypt distension and mucus hypersecretion in the colon
• crypts of the jejunum and ileum are filled with excessive mucus, indicating gastrointestinal mucus accumulation
• variable penetrance of intestinal obstruction in the jejunum and meconium blockage of ileum (meconium ileus) (J:2584)
• death caused by severe peritonitis

endocrine/exocrine glands
• dilation and blockage of pancreatic ducts in 5 of 10 mutants aged 17 days or older
• crypts of Lieberkuhn are severely dilated with mucus
• acinar dilation although no obvious accumulation of mucu

growth/size
• those surviving past 2-5 days of age are runted and half the weight of wild-type

immune system
• death caused by severe peritonitis
• several mutants develop eye infections and are prone to persistent eye closure

vision/eye
• acinar dilation although no obvious accumulation of mucu

respiratory system
N
• no accumulation of mucus in the lungs (J:23850)

Mouse Models of Human Disease
OMIM IDRef(s)
Cystic Fibrosis; CF 219700 J:2584