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Phenotypes Associated with This Genotype
Genotype
MGI:5445419
Allelic
Composition
Cftrtm1Eur/Cftrtm1Eur
Genetic
Background
involves: 129P2/OlaHsd * FVB/N
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Mouse lines carrying:
Cftrtm1Eur mutation (0 available); any Cftr mutation (13 available)
phenotype observed in females
phenotype observed in males
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 (J:28979)
• however, no distention of the crypts in the small intestine or complete intestinal obstruction either in the ileum or colon is seen (J:28979)
• crypts in the colon show some mucus retention and slight dilatation and have a layer of thick mucus on top of the crypts (J:28979)
• however, no distention of the crypts in the small intestine or complete intestinal obstruction either in the ileum or colon is seen (J:28979)
• focal hypertrophy of goblet cells in the crypts of Lieberkuhn, predominantly seen in deep crypts (J:28979)
• crypts containing hypertrophic goblet cells are located in clusters (J:28979)
• however, the majority of the intestine appears normal (J:28979)
• focal hypertrophy of goblet cells in the crypts of Lieberkuhn, predominantly seen in deep crypts (J:28979)
• crypts containing hypertrophic goblet cells are located in clusters (J:28979)
• however, the majority of the intestine appears normal (J:28979)
• 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 (J:28979)
• luminal hyperpolarization induced by forksolin is reduced, whereas the response to glucose addition which activates the sodium/glucose co-transporter is normal (J:28979)
• 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 (J:28979)
• luminal hyperpolarization induced by forksolin is reduced, whereas the response to glucose addition which activates the sodium/glucose co-transporter is normal (J:28979)

respiratory system
• basal in vivo nasal potential difference is higher than in controls (J:28979)
• nasal potential increases in response to a large chloride gradient created by substitution of chloride by gluconate in the superfusion solution (J:28979)
• basal in vivo nasal potential difference is higher than in controls (J:28979)
• nasal potential increases in response to a large chloride gradient created by substitution of chloride by gluconate in the superfusion solution (J:28979)

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 (J:28979)
• however, no distention of the crypts in the small intestine or complete intestinal obstruction either in the ileum or colon is seen (J:28979)
• crypts in the colon show some mucus retention and slight dilatation and have a layer of thick mucus on top of the crypts (J:28979)
• however, no distention of the crypts in the small intestine or complete intestinal obstruction either in the ileum or colon is seen (J:28979)

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 (J:28979)
• 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 (J:28979)

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


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory