Mouse Genome Informatics
hm
    Cftrtm1Hgu/Cftrtm1Hgu
involves: 129P2/OlaHsd * MF1
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• 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

homeostasis/metabolism
• 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 (J:14614)

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