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Allele Symbol Allele Name Allele ID |
Zc3h12atm1c(EUCOMM)Hmgu targeted mutation 1c, Helmholtz Zentrum Muenchen GmbH MGI:6115370 |
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| Summary |
2 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice show the presence of robust myeloid cell-driven systemic inflammation
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| N |
• features of primary biliary cholangitis are not seen in mice
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice show the eventual disruption of the bile duct epithelium
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• extensive bile duct hyperplasia at 6 weeks of age
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• 6 to 52-week-old mice show extensive parenchymal inflammation
• mice of all ages show increased leukocyte infiltration into the liver parenchyma; the predominant population of leukocytes is T lymphocytes in 6-week-old-mice, while 24-week-old mice show an additional accumulation of B lymphocytes, macrophages and neutrophils
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• 6 to 52-week-old mice show extensive parenchymal inflammation and parenchymal fibrosis
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• 6 to 52-week-old mice show parenchymal fibrosis and fibrosis in portal areas
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• active and progressive proliferation of intrahepatic bile ducts is seen in 6 to 52-week-old mice, with intrahepatic and periportal cholangiocyte proliferation
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• symptoms of primary biliary cholangitis are already seen at P10; intrahepatic bile duct pathology is extensive at 6 weeks, less severe in the liver parenchyma at 24 weeks, and modestly reintensified by 52 weeks
• males and females show the same features of primary biliary cholangitis
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• mice show bile duct lumen obstruction due to proliferation of bile ducts, bile duct epithelium disruption, inflammation, and fibrosis
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• mice show the eventual disruption of the bile duct epithelium
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• extensive bile duct hyperplasia at 6 weeks of age
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• IgG concentrations are increased at 6- and 24-weeks of age
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• IgM concentrations are increased at 6- and 24-weeks of age
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• alkaline phosphatase activity in the serum is increased at 6 weeks of age
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• concentration of total bile acids in the serum is increased at 6 weeks of age
• bile acid deposition in hepatocytes eventually occurs
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• IgG concentrations are increased at 6- and 24-weeks of age
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• IgM concentrations are increased at 6- and 24-weeks of age
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• 60-90% mice exhibit increased plasma concentrations of anti-PDC-E2 mitochondrial autoantibodies
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• 90% of 12-week-old mice exhibit elevated anti-gp-210 nuclear autoantibodies
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• eventually mice show formation of small granulomas in the liver
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• symptoms of primary biliary cholangitis are already seen at P10; intrahepatic bile duct pathology is extensive at 6 weeks, less severe in the liver parenchyma at 24 weeks, and modestly reintensified by 52 weeks
• males and females show the same features of primary biliary cholangitis
|
|
• 6 to 52-week-old mice show extensive parenchymal inflammation
• mice of all ages show increased leukocyte infiltration into the liver parenchyma; the predominant population of leukocytes is T lymphocytes in 6-week-old-mice, while 24-week-old mice show an additional accumulation of B lymphocytes, macrophages and neutrophils
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| primary biliary cholangitis | DOID:12236 |
OMIM:PS109720 |
J:304105 | |
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 05/07/2024 MGI 6.23 |
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