Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cftrtm1Hgu mutation
(0 available);
any
Cftr mutation
(97 available)
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Generation 27 and 28 Cftrtm1Hgu/Cftrtm1Hgu mice exhibit focal hypertrophy of goblet cells
mortality/aging
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• Background Sensitivity: in early generations, 8-10% die of intestinal obstruction around weaning, survival is 76% at 3 months of age for the F8-F10 generations, early deaths are still seen at F18, but by the 25th generation, no increased mortality over wild-type is seen
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digestive/alimentary system
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• F28 mice show focal hypertrophy of goblet cells in the ileum, but not in the jejunum
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• Background Sensitivity: 8-10% of mutants in the early generations die of intestinal obstruction at weaning, however later generations do not exhibit intestinal blockage
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reproductive system
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N |
• males and females are fertile
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cellular
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• F28 mice show focal hypertrophy of goblet cells in the ileum, but not in the jejunum
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cftrtm1Hgu mutation
(0 available);
any
Cftr mutation
(97 available)
|
|
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Generation 27 and 28 Cftrtm1Hgu/Cftrtm1Hgu mice exhibit focal hypertrophy of goblet cells
mortality/aging
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• Background Sensitivity: in early generations, 8-10% die of intestinal obstruction around weaning, survival is 76% at 3 months of age for the F8-F10 generations, early deaths are still seen at F18, but by the 25th generation, no increased mortality over wild-type is seen
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digestive/alimentary system
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• F27 mice show focal hypertrophy of goblet cells in the ileum, but not in the jejunum
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• Background Sensitivity: 8-10% of mutants in the early generations die of intestinal obstruction at weaning, however later generations do not exhibit intestinal blockage
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reproductive system
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N |
• males and females are fertile
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cellular
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• F27 mice show focal hypertrophy of goblet cells in the ileum, but not in the jejunum
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|
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cftrtm1Hgu mutation
(0 available);
any
Cftr mutation
(97 available)
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immune system
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• mutants exposed to S. aureus exhibit increased lymphoid infiltrate and bronchiolitis compared to wild-type
• mutants exposed to B. cepacia exhibit much more severe bronchopneumonia and mixed inflammatory infiltrate than wild-type
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• mutants are unable to clear Staphylococcus aureus and Burkholderia cepacia from the lungs and develop lung disease after repeated bacterial exposure
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respiratory system
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N |
• under normal conditions (without bacterial exposure), no lung abnormalities are detected in homozygotes
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• mutants exposed to S. aureus exhibit increased lymphoid infiltrate and bronchiolitis compared to wild-type
• mutants exposed to B. cepacia exhibit much more severe bronchopneumonia and mixed inflammatory infiltrate than wild-type
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• mutants exhibit lung disease in response to repeated bacterial exposure
• mutants exhibit increased lymphoid infiltrate, goblet cell hyperplasia, mucus retention in the airways, bronchiolitis compared to wild-type after S. aureus infection
• mutants exhibit severe bronchopneumonia and mucus retention in the major airways, goblet cell hyperplasia and metaplasia, massive mixed inflammatory infiltrate, obliteration of smaller airways, and extensive oedema compared to wild-type after B. cepacia infection
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• mutants exposed to bacteria (S. aureus and B. cepacia) exhibit increased goblet cell hyperplasia compared to wild-type
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cftrtm1Hgu mutation
(0 available);
any
Cftr mutation
(97 available)
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mortality/aging
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• 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
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digestive/alimentary system
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• mild colon dilatation with abnormal mucus accumulation
• distension of epithelial cells, most prominent in the crypts
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• approximately 5% incidence of fatal meconium ileus
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• chloride ion transport is altered as indicated by reduced rectal and colonic/caecal potential differences
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respiratory system
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• one mutant at P30 showed mild focal pulmonary atelectasis, with mucus obstruction of a small bronchus
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• 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
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reproductive system
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• one male exhibited increased mucin in the vas deferens
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Analysis Tools