Analysis Tools
mortality/aging
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• incomplete penetrance, likely due to renal failure from SLE-like disease
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neoplasm
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• correlated with proliferative phenotype seen in B cells
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hematopoietic system
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• unimmunized mice at 2 months displayed numerous splenic germinal centers, suggestive of active, proliferative state
• increased B cell proliferation via the immunoglobulin receptor in vitro
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• increased T cell receptor mediated proliferation, but T cell development normal
• normal T cell-dependent immune responses, as assessed by antibody titers after immunization with TNP-OVA
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• evident between 3 and 6 months of age
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• concomitant 50% decrease in long-lived recirculating bone marrow B cells
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• 50% increase in the number of pre-B cells in the bone marrow compared to controls
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• small, 17% decrease in mature naive B cells
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• increased numbers of large, well developed germinal centers evident at 6 weeks of age
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• ill-defined marginal zone evident at 6 weeks of age
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homeostasis/metabolism
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• evident in 7 of 24 3 to 5 month old mice
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• evident in 7 of 24 3 to 5 month old mice
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• incomplete penetrance, evident in several mice, indicative, along with increased creatinine and urea in serum, of renal failure
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immune system
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• unimmunized mice at 2 months displayed numerous splenic germinal centers, suggestive of active, proliferative state
• increased B cell proliferation via the immunoglobulin receptor in vitro
|
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• increased T cell receptor mediated proliferation, but T cell development normal
• normal T cell-dependent immune responses, as assessed by antibody titers after immunization with TNP-OVA
|
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• evident between 3 and 6 months of age
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• concomitant 50% decrease in long-lived recirculating bone marrow B cells
|
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• 50% increase in the number of pre-B cells in the bone marrow compared to controls
|
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• small, 17% decrease in mature naive B cells
|
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• increased numbers of large, well developed germinal centers evident at 6 weeks of age
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• ill-defined marginal zone evident at 6 weeks of age
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• SLE-like phenotype seen
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• deposition of IgG, IgM, and C3 in glomeruli
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• autoantibodies reactive with kidney sections of wild-type mice were detected in 5 or 6 week old mutant mice, but not in controls
(J:50626)
• increased autoantibody (anti-ANA, anti-ssDNA, anti-dsDNA, anti-histone) concentrations in serum, especially in female mice
(J:81805)
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• hypercellularity, lobularity, segmental sclerosis, and enlarged glomeruli, caused by immune complex deposits
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renal/urinary system
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• incomplete penetrance, evident in several mice, indicative, along with increased creatinine and urea in serum, of renal failure
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• hypercellularity, lobularity, segmental sclerosis, and enlarged glomeruli, caused by immune complex deposits
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cellular
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• unimmunized mice at 2 months displayed numerous splenic germinal centers, suggestive of active, proliferative state
• increased B cell proliferation via the immunoglobulin receptor in vitro
|
|
• increased T cell receptor mediated proliferation, but T cell development normal
• normal T cell-dependent immune responses, as assessed by antibody titers after immunization with TNP-OVA
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growth/size/body
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• evident between 3 and 6 months of age
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| systemic lupus erythematosus | DOID:9074 |
OMIM:152700 OMIM:300809 OMIM:605480 OMIM:608437 OMIM:609903 OMIM:609939 OMIM:610065 OMIM:610066 OMIM:612254 OMIM:612378 OMIM:613145 OMIM:614420 |
J:50626 , J:81805 | |
