Mouse Genome Informatics
hm
    Cftrtm1Eur/Cftrtm1Eur
involves: 129P2/OlaHsd * FVB/N
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
growth/size/body

digestive/alimentary system
• crypts in the colon show some mucus retention and slight dilatation and have a layer of thick mucus on top of the crypts
• however, no distention of the crypts in the small intestine or complete intestinal obstruction either in the ileum or colon is seen
• focal hypertrophy of goblet cells in the crypts of Lieberkuhn, predominantly seen in deep crypts
• crypts containing hypertrophic goblet cells are located in clusters
• however, the majority of the intestine appears normal
• in the ileum, the initial potential differences and equivalent short circuit currents (Ieq) are lower, indicating that cAMP-induced activation of calcium-dependent chloride channels is reduced in the ileum
• luminal hyperpolarization induced by forksolin is reduced, whereas the response to glucose addition which activates the sodium/glucose co-transporter is normal

respiratory system
• basal in vivo nasal potential difference is higher than in controls
• nasal potential increases in response to a large chloride gradient created by substitution of chloride by gluconate in the superfusion solution

endocrine/exocrine glands
• crypts in the colon show some mucus retention and slight dilatation and have a layer of thick mucus on top of the crypts
• however, no distention of the crypts in the small intestine or complete intestinal obstruction either in the ileum or colon is seen

liver/biliary system
• mutants show a reduced potential difference response to forksolin in the gallbladder, indicating that cAMP-induced activation of calcium-dependent chloride channels is reduced in the gallbladder

Mouse Models of Human Disease
OMIM IDRef(s)
Cystic Fibrosis; CF 219700 J:28979