Analysis Tools
mortality/aging
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• Background Sensitivity: half of homozygous mice die in the first week of age, with the remainder dying between 25- 35 days of age
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Allele Symbol Allele Name Allele ID |
Cftrtm1Hsc targeted mutation 1, Hospital for Sick Children MGI:1857545 |
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| Summary |
6 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• Background Sensitivity: half of homozygous mice die in the first week of age, with the remainder dying between 25- 35 days of age
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• jejuna obtained from class III mice (those surviving past three weeks of age) exhibit a negative basal potential difference across the intestinal epithelium, although it is less negative than that observed in jejuna from control animals
• transepithelial resistance in the jejunum of CIII mice (those surviving past three weeks of age) was higher than that seen in control animals
• the chloride channel blocker NPPB inhibited the jejunal PD measured in solid diet- and liquid diet-fed control animals to a similar extent, suggesting that the jejunal epithelium of class III mice can produce chloride secretion in the absence of Cftr
• the value of the negative potential difference across the jejunal intestinal epithelium correlates with survival in class II (survive only to weaning age) and class III (survive past 6 weeks of age) mice
• the chloride channel blocker NPPB effectively inhibits transepithelial PD in both class II and class III mice
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• Background Sensitivity: most homozygotes die between birth and 25 days of age (class II mice); 31% survive longer than 6 weeks of age (class III mice)
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• Background Sensitivity: on this mixed genetic background, most homozygotes die between birth and 25 days of age (class I and II mice); 31% survive longer than 6 weeks of age (class III mice)
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• the chloride channel blocker NPPB inhibited the jejunal PD measured in solid diet- and liquid diet-fed control animals to a similar extent, suggesting that the jejunal epithelium of class III mice can produce chloride secretion in the absence of Cftr
• the value of the negative potential difference across the jejunal intestinal epithelium correlates with survival in class II (survive only to weaning age) and class III (survive past 6 weeks of age) mice
• the chloride channel blocker NPPB effectively inhibits transepithelial PD in both class II and class III mice, suggesting that other channels can mediate transepithelial chloride transport in mutant Cftr mice
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• Background Sensitivity: most homozygotes survive to 20 days of age with the majority dying between 20 and 35 days of age (class II mice); 25% survive longer than 6 weeks of age (class III mice)
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• increased intestinal mucosal thickness in small intestine and colon
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• increase in goblet cell population in small intestine and colon
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• accumulation of secreted mucus in crypt lumina
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• increase in goblet cell population in small intestine and colon
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• severe intestinal obstruction in class I mice (die in first week of life)
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• severe intestinal obstruction in class I mice by mucus containing plug
• class II mice exhibit severe intestinal obstruction by mucus containing plug, with the ileum and jejunum the major sites of mucus accumulation with obstructive accumulation
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• absence of cAMP-induced Cl- conductance in crypt cells isolated from ileal mucosa
• the A23187 calcium ionophore elicited an calcium-dependent Cl-selective current in ileal crypt cells in contrast to control cells
• A23187 calcium ionophore elicited a greater change in anion permeability in ileal crypts than in controls in mice on this genetic background, suggesting partial compensation of ion transport abnormalities caused by absence of Cftr in class III mice that survive past 6 weeks of age
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• accumulation of secreted mucus in crypt lumina
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• increase in goblet cell population in small intestine and colon
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• smaller than control littermates at 2-3 weeks of age compared to controls, but this difference was not seen at 8-12 weeks of age
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• animals lose weight in the days preceding death
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• Class I mice do not gain weight after birth
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• Background Sensitivity: death in the first week of life (class I mice) or by weaning (class II mice), incomplete penetrance
• Background Sensitivity: 29.7% survive past 6 weeks of age (class III mice)
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• nasal potential difference in homozygotes is greater than controls
• greater amelioride-sensitive component of nasal potential difference suggests an increased sodium ion transport capacity in nasal epithelia
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• increase in goblet cell population in small intestine and colon
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• increase in goblet cell population in small intestine and colon
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| cystic fibrosis | DOID:1485 |
OMIM:219700 |
J:31759 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• Background Sensitivity: most mice die in the first week of age or prior, since homozygotes are underrepresented at genotyping age; none survive past 25 days
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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 07/02/2024 MGI 6.13 |
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