Analysis Tools
mortality/aging
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• 33% of mice die between 10 and 12 months of age
• likely cause of death is glomerulonephritis
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growth/size/body
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• decreased body weight is observed in mice starting at 6 months of age
• mice that survive to 12 months of age are smaller than controls
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immune system
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• double-positive thymocytes have CD5 and CD69 upregulated and are hyper-responsive to in vitro stimulation
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• T1 and T2 B cell numbers are increased in the spleen similar to mice homozygous for just the Ptprctm1Weis allele
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• follicular B cell numbers are reduced by about a third compared to controls
• a similar phenotype is observed in mice homozygous for just the Ptprctm1Weis allele
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• plasma cell numbers in the spleen is increased 4-fold at 3 months of age compared to controls
• a similar phenotype is observed in mice homozygous for just the Ptprctm1Weis allele
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• shift in ratio is evident at six weeks of age and lasts through 12 months of age
• the CD4:CD8 T cell ratio in the periphery is over 2.5 at six months of age
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• CD44hi CD62Llo memory T cells are increased about 3-fold at 6 months of age compared to controls
• differences are noticeable at 2 months of age with increases progressing with age
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• an increased percentage of B cells express the activation marker CD69 in vivo at 6 months of age
• a larger percentage of B cells express CD69 than controls in response to stimulation in vitro
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• follicular B cells are hyper-responsive to stimulation in vitro
• a similar phenotype is observed in mice homozygous for just the Ptprctm1Weis allele
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• the activation marker CD69 is upregulated on both CD4 and CD8 T cells in vivo
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• double-positive thymocytes are hyper-responsive to in vitro stimulation
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• lymphadenopathy is observed as early as 8 weeks of age
• is progressive with time and greatly exceeds the mild lymphoproliferation evident in each of the single mutants
• cell counts are variable with some counts being 10-fold higher than controls at six months of age
• lymphadenopathy is generally out of proportion to the degree of splenomegaly observed
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• auto-IgG antibodies are detected in some mice starting at 3 months of age
• by 9 months of age, auto-antibodies titers can reach levels observed in MRL/Lpr mice
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• perivascular infiltrates occur in the liver of mice over 12 months of age
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• kidneys of 12 month old mice have perivascular lymphocytic infiltrates, interstitial lymphocytic infiltrates, and hypercellular glomeruli with evidence of segmental sclerosis
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• perivascular infiltrates occur in the lung of mice over 12 months of age
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renal/urinary system
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• mice over 12 months of age have variable degrees of proteinuria
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• kidneys of 12 month old mice have perivascular lymphocytic infiltrates, interstitial lymphocytic infiltrates, and hypercellular glomeruli with evidence of segmental sclerosis
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• kidneys of 12 month old mice have hypercellular glomeruli with evidence of segmental sclerosis
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pale kidney
(
J:147129
)
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• kidneys from mice over 12 months of age are pale and nodular
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homeostasis/metabolism
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• mice over 12 months of age have variable degrees of proteinuria
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liver/biliary system
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• perivascular infiltrates occur in the liver of mice over 12 months of age
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respiratory system
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• perivascular infiltrates occur in the lung of mice over 12 months of age
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hematopoietic system
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• double-positive thymocytes have CD5 and CD69 upregulated and are hyper-responsive to in vitro stimulation
|
|
• T1 and T2 B cell numbers are increased in the spleen similar to mice homozygous for just the Ptprctm1Weis allele
|
|
• follicular B cell numbers are reduced by about a third compared to controls
• a similar phenotype is observed in mice homozygous for just the Ptprctm1Weis allele
|
|
• plasma cell numbers in the spleen is increased 4-fold at 3 months of age compared to controls
• a similar phenotype is observed in mice homozygous for just the Ptprctm1Weis allele
|
|
• the CD4:CD8 T cell ratio in the periphery is over 2.5 at six months of age
• shift in ratio is evident at six weeks of age and lasts through 12 months of age
|
|
• CD44hi CD62Llo memory T cells are increased about 3-fold at 6 months of age compared to controls
• differences are noticeable at 2 months of age with increases progressing with age
|
|
• an increased percentage of B cells express the activation marker CD69 in vivo at 6 months of age
• a larger percentage of B cells express CD69 than controls in response to stimulation in vitro
|
|
• follicular B cells are hyper-responsive to stimulation in vitro
• a similar phenotype is observed in mice homozygous for just the Ptprctm1Weis allele
|
|
• the activation marker CD69 is upregulated on both CD4 and CD8 T cells in vivo
|
|
• double-positive thymocytes are hyper-responsive to in vitro stimulation
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endocrine/exocrine glands
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• double-positive thymocytes are hyper-responsive to in vitro stimulation
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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:147129 | |
