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Phenotypes Associated with This Genotype
Genotype
MGI:2177543
Allelic
Composition
Cftrtm3Hgu/Cftrtm3Hgu
Genetic
Background
involves: 129P2/OlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cftrtm3Hgu mutation (0 available); any Cftr mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
digestive/alimentary system
• dilated intestinal mucus glands
• presence of eosinophilic concretions in the crypts of Lieberkuhn resulting in dilation, particularly in the jejunum
• small intestines show loss of villi with accumulation of necrotic and fecal material in the lumen
• salivary gland appeared hypercellular due to serous cells losing the vacuolated appearance
• 33% die early from intestinal obstruction
• obstructions were found distal or proximal to the caecum
• baseline PD values and response to forskolin were significantly reduced in the jejunum, cecum and rectum
• obstructions were found distal or proximal to the caecum

endocrine/exocrine glands
N
• no difference in the acinar or epithelial cells of the pancreas
• presence of eosinophilic concretions in the crypts of Lieberkuhn resulting in dilation, particularly in the jejunum
• salivary gland appeared hypercellular due to serous cells losing the vacuolated appearance
• one animal showed focal biliary cirrhosis, with biliary ducts absent and replaced with fibrotic stroma and inflammatory infiltrate
• 3/15 animals showed hyperplasia of the bile duct epithelia
• inspissated eosinophilic material in the lumen of the pharyngeal submucosal glands
• gallbladder appears black and enlarged

growth/size/body
• homozygous mice are runted
• homozygous mice weigh only 50 to 70% of control siblings

immune system
• obstructions were found distal or proximal to the caecum
• 5/9 gallbladders showed a presence of large numbers of polymorphonuclear cells, particularly eosinophils
• 2/9 gallbladder walls had a vacuolated appearance with inflammatory cells present

liver/biliary system
• one animal showed focal biliary cirrhosis, with biliary ducts absent and replaced with fibrotic stroma and inflammatory infiltrate
• 3/15 animals showed hyperplasia of the bile duct epithelia
• gallbladder appears black and enlarged
• 5/9 gallbladders showed a presence of large numbers of polymorphonuclear cells, particularly eosinophils
• 2/9 gallbladder walls had a vacuolated appearance with inflammatory cells present
• one animal showed bile pigments in the hepatocytes

mortality/aging
• only 27% of homozygous animals survive to 35 days of age

reproductive system
N
• reproductive tract appears normal in all animals examined

respiratory system
N
• no morphological abnormalities were detected in the lung, tracheal epithelium or pharyx
• baseline potential difference (PD) and response to forskolin were significantly increased in nasal epithelium
• baseline potential difference (PD) and response to amiloride were significantly increased in nasal epithelium

Mouse Models of Human Disease
OMIM ID Ref(s)
Cystic Fibrosis; CF 219700 J:32766


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
05/17/2016
MGI 6.03
The Jackson Laboratory