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Phenotypes Associated with This Genotype
involves: 129P2/OlaHsd * MF1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cftrtm1Hgu mutation (0 available); any Cftr mutation (13 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
• low incidence of lethality due to intestinal blockage; 5% die within 1 week of birth and 2% die around weaning, with 93% surviving to adulthood

• altered chloride ion transport in respiratory and gastrointestinal tracts

digestive/alimentary system
• mild colon dilatation with abnormal mucus accumulation
• distension of epithelial cells, most prominent in the crypts
• chloride ion transport is altered as indicated by reduced rectal and colonic/caecal potential differences
• approximately 5% incidence of fatal meconium ileus

respiratory system
• chloride ion transport in the nose is defective as indicated by an increased nasal potential difference, but no difference is seen throughout the lower airways below the larynx
• perfusion of the trachea with a low-chloride solution shows a reduction in resultant hyperpolarization
• one mutant at P30 showed mild focal pulmonary atelectasis, with mucus obstruction of a small bronchus

reproductive system
• one male exhibited increased mucin in the vas deferens

Mouse Models of Human Disease
OMIM ID Ref(s)
Cystic Fibrosis; CF 219700 J:14614

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
MGI 6.01
The Jackson Laboratory