digestive/alimentary system
endocrine/exocrine glands
immune system
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• B cell turnover over a one week period is twice that of controls
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• immature B cell numbers in the spleen are reduced by about a third
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• there is a 2- to 4- fold reduction in the number of mature recirculating B cells found in the bone marrow
• the IgMlowIgD+ mature B cell population is strongly diminished in the spleen by 3- to 4- fold
• B cell numbers in the lymph nodes are also strongly reduced
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• B-1 B cell numbers are strongly decreased in the peritoneum cavity
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• follicular B cells are the most diminished B cell population in the spleen
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• marginal zone B cell numbers are reduced about 4-fold
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• B cell turnover over a one week period is twice that of controls
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• immature B cell numbers in the spleen are reduced by about a third
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• there is a 2- to 4- fold reduction in the number of mature recirculating B cells found in the bone marrow
• the IgMlowIgD+ mature B cell population is strongly diminished in the spleen by 3- to 4- fold
• B cell numbers in the lymph nodes are also strongly reduced
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• B-1 B cell numbers are strongly decreased in the peritoneum cavity
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• follicular B cells are the most diminished B cell population in the spleen
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• marginal zone B cell numbers are reduced about 4-fold
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• B cell turnover over a one week period is twice that of controls
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• there is a pronounced block in B cell development at transition stage 1 in the spleen
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• mice show dramatic reduction in peripheral B cell numbers
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• numbers of mature B cells are reduced to 10% of levels in controls
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• there is a pronounced block in B cell development at transition stage 1 in the spleen
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• mice show dramatic reduction in peripheral B cell numbers
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• numbers of mature B cells are reduced to 10% of levels in controls
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• increase in the numbers of apoptotic CD8+/HASlow/- and CD4+/HASlow/- mature thymocytes
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• spleen and lymph nodes are nearly devoid of T cells
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• only a mild reduction in the CD4 single-positive cells in the thymus
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• 50% reduction in CD8 single-positive cells in the thymus
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• loss of single-positive thymocytes during final maturation stages in the thymus
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• increase in the numbers of apoptotic CD8+/HASlow/- and CD4+/HASlow/- mature thymocytes
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• spleen and lymph nodes are nearly devoid of T cells
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• only a mild reduction in the CD4 single-positive cells in the thymus
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• 50% reduction in CD8 single-positive cells in the thymus
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• loss of single-positive thymocytes during final maturation stages in the thymus
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• increase in the numbers of apoptotic CD8+/HASlow/- and CD4+/HASlow/- mature thymocytes
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• 8 week old mice show signs of steatohepatitis in the liver, characterized by immune cell infiltration into the parenchyma
• in 8-week old mice, hepatocytes show features of large-cell dysplasia with strong anisokaryosis; large areas of irregularly shaped glycogen deposits are detected
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• at 6 months, livers show macroscopic nodules
• focal areas of lipid accumulation and low-grade dysplastic nodules with expansive growth are observed
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• at 6 months, hepatocytes show lipid deposition and ballooning
• in 8-week old mice, hepatocytes show features of large-cell dysplasia with strong anisokaryosis; large areas of irregularly shaped glycogen deposits are detected
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• mitochondria of hepatocytes appear swollen and display a reduction in mitochondrial crests compared to wild-type at 8 weeks of age
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• at 6 months, mice show signs of steatosis and increased lipid deposition in hepatocytes
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• multiple tumors are found in livers of 12-month old males; malignant liver tumors are present in all mice examined
• average number of tumors per mouse is 5.75 with average size of 8.58 mm
• tumors show expansive growth, increased cellularity, broad trabecular growth pattern in 4 to 6 layers, eosinophilia, higher cytoplasmic index compared to non malignant areas, and complete absence of portal tracts
• tumors show increased proliferation and higher mitotic indices (~1.44 per high-power field)
• tumors at 9 months show dysplastic foci and nodules
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• multiple tumors are found in livers of 12-month old males; malignant liver tumors are present in all mice examined
• average number of tumors per mouse is 5.75 with average size of 8.58 mm
• tumors show expansive growth, increased cellularity, broad trabecular growth pattern in 4 to 6 layers, eosinophilia, higher cytoplasmic index compared to non malignant areas, and complete absence of portal tracts
• tumors show increased proliferation and higher mitotic indices (~1.44 per high-power field)
• tumors at 9 months show dysplastic foci and nodules
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• cytokine expression is upregulated in mutants
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• upon LPS injection, NF kappa b activation is completely absent
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• following exposure to LPS, mice exhibit increased liver failure and hepatocyte apoptosis compared to similarly treated wild-type mice
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• 8 week old mice show signs of steatohepatitis in the liver, characterized by immune cell infiltration into the parenchyma
• in 8-week old mice, hepatocytes show features of large-cell dysplasia with strong anisokaryosis; large areas of irregularly shaped glycogen deposits are detected
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• mitochondria of hepatocytes appear swollen and display a reduction in mitochondrial crests compared to wild-type at 8 weeks of age
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• 8-week old mice show an increase in apoptotic cells in the liver
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• livers show increased proliferation
• at 8 weeks, there is a significant increase in progenitor cells and activation of oval cells in the liver
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• mice show a decrease in spontaneous serum glucose levels compared to controls
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• mice show elevated serum levels of alanine aminotransferase (ALT) at 3 weeks, indicative of hepatocyte damage; ALT levels are further increased at 8 weeks
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• mice show increased JNK kinase activity
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
steatotic liver disease | DOID:9452 |
OMIM:228100 |
J:118336 |
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• spontaneous serum ALT levels are decreased compared to single Ikbkg mutants at 8 weeks of age
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• mice show increased JNK kinase activity
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• completely absent compared to single Ikbkg mutants at 8 weeks of age
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N |
• liver inflammation, hepatocyte apoptosis, increased hepatocyte proliferation, and focal lipid accumulation are inhibited in mutants compared to Ikbkg single mutants
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• completely absent compared to single Ikbkg mutants at 8 weeks of age
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• double mutants are protected from LPS-induced hepatocyte apoptosis
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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 11/12/2024 MGI 6.24 |
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