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Phenotypes Associated with This Genotype
Genotype
MGI:2177534
Allelic
Composition
Cftrtm2Cam/Cftrtm2Cam
Genetic
Background
involves: 129S/SvEv
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cftrtm2Cam mutation (1 available); any Cftr mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 35% of homozygous mice are dead by 16 days of age
• mutant mice surviving past weaning age die by 6-8 months of age

digestive/alimentary system
• crypt of Lieberkuhn distension and mucus hyper-accumulation in the colon in 9/11 animals
• dilated crypts of Lieberkuhn in jejunum and ileum with excessive mucus accumulation in 9/11 mutant mice
• inspissated meconium obstructions of the small intestine which caused rupture and peritonitis
• the basal SCC in the mutant colon epithelium is smaller than controls
• forskolin reduced and often reversed the resting SCC value, with responses to forsolin, carbachol and frusemide all significantly different from controls, independent of animal age

immune system

endocrine/exocrine glands
N
• no significant differences in mutant pancreas compared with controls
• no significant differences in mutant lacrymal glands compared with controls
• crypt of Lieberkuhn distension and mucus hyper-accumulation in the colon in 9/11 animals
• dilated crypts of Lieberkuhn in jejunum and ileum with excessive mucus accumulation in 9/11 mutant mice

respiratory system
N
• no pathological changes were seen in mutant lung compared with controls
• the amiloride sensitive current in mutant and control tracheas were similar, suggesting normal sodium ion transport
• basal short circuit current level in the mutant tracheal epithelium is not significantly different from controls
• the forskolin sensitive current in mutant trachea is reduced compared to controls, suggesting a defect in chloride transport; this difference is greater in young animals (18-32 days of age) vs. older animals, where the difference is not significant
• the A23187 sensitive current in mutant in mutant trachea is similar at all ages compared to controls

Mouse Models of Human Disease
OMIM ID Ref(s)
Cystic Fibrosis; CF 219700 J:27734


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
07/19/2016
MGI 6.04
The Jackson Laboratory