Analysis Tools
immune system
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• 2.2-fold increase in number of proliferating CD4-positive T cells isolated from spleen and lymph nodes
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• is present in some mice by 3 months of age
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• after 4 weeks, mice exhibit high levels of eosinophils
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• severe but partial block in development of DN alpha-beta T cells into DP T cells
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• the number of double positive cells decrease after birth and are almost undetectable by 7 weeks of age
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• gamma-delta T cell development is unaffected in these mice
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• at 6 weeks, the number of B cells in secondary lymphoid organs is increased 7 to 10 times compared to wild-type
• B cells in enlarged secondary lymphoid organs exhibit 25% hyperactivated phenotype, 50% antibody-producing phenotype and 25% a resting phenotype
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• 500-fold increase in plasma cells in the spleen and lymph nodes of 6 week old mice
• 5% of total lymph node cells are plasma cells by 6 weeks of age
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• the ratio of CD4 to CD8 cells is skewed towards CD4 cells
• the ratio of single positive T cells in the secondary lymphoid organs is bias towards CD4+ cells and this population expands over time
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• the number of CD4+ cells increases due to increased proliferation and extended survival
(J:77098)
• are first observed in secondary lymph nodes at 4 weeks of age
(J:125722)
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• during the first weeks of life, mice develop a Th2 lymphoproliferative disorder
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• the majority of T cells found in the periphery have cell surface markers of memory T cells (low TCR expression, CD44-high, CD62L-low, CD69-positive)
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• 25% of B cells from secondary lymphoid organs have cell surface markers indicative of a hyperactive state
• an additional 50% of B cells are activated compared to B cells from wild-type mice that almost exclusively in a resting state
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• wild-type B cells secrete abnormally high amounts of IgG1 when cultured with mutant CD4 T cells
• this effect is dependent on IL-4
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• thymus cellularity is one tenth of wild-type
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• spleen and lymph nodes start to enlarge at 7 weeks of age
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• spleen weight becomes higher after 6 weeks of age
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• spleen cellularity is increased 5 times compared to in wild-type mice
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• germinal centers form in every B cell follicle starting at 4 weeks of age
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• are pale and have accumulations of plasma and immunoblast cells
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• architecture of lymph node follicles becomes disrupted by 3 months of age due to large number of activated B cells
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• germinal centers form in every B cell follicle starting at 4 weeks of age
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• spleen and lymph nodes start to enlarge at 7 weeks of age
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• up to 10,000 times wild-type
(J:77098)
• adult mice have serum levels of IgE that are 10,000 times greater than normal
(J:125722)
• antibodies are polyclonal
(J:125722)
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• up to 200 times wild-type
(J:77098)
• adult mice have serum levels that are 200 times greater than normal
(J:125722)
• antibodies are polyclonal
(J:125722)
• serum IgG1 levels remain elevated in mice treated with corticosteroid at 4 or 7-8 weeks of age
(J:262891)
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• 6-fold greater levels in the sera of adult mice
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• 5.4% of CD4 T cells from lymph node produce INF-gamma upon stimulation as compared to 0.3% of CD4 T cells from wild-type mice
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• 79.2% of CD4 T cells from lymph node produce IL-4 upon stimulation as compared to 0.3% of CD4 T cells from wild-type mice
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• detectable at 2 weeks of age
• levels increase with age
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• detectable at 2 weeks of age
• 17-fold greater concentrations than wild-type mice in adult mice
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• detectable at 2 weeks of age
• 100-fold greater concentrations than wild-type mice in adult mice
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• the lung, liver, and kidney have prominent lymphocytic infiltrations
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• all mice exhibit organ inflammatory lesions with infiltration of mononuclear inflammatory cells starting from 6 weeks of age
• infiltrations of plasma cells, CD4+ T cells, and macrophages into the inflammatory lesions
• infiltrating plasma cells express IgG, predominantly IgG1
• mice treated with corticosteroid at 4 weeks of age up to 6 weeks of age show inhibition of the development of lesions in the salivary glands and pancreas, but not in the kidneys
• mice treated with corticosteroid from 7-8 weeks of age when they already have inflammatory lesions to 9-10 weeks of age, show lowered inflammation
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• mice develop phlebitis, with vascular lesions in the pancreas of 10% and 11.1% of 10 and 20 week old mice and obliterating phlebitis in lungs of 70% and 77.8% of 10 and 20 week old mice, respectively
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• multiple inflammatory lesions are seen around the ducts in the salivary glands
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• multiple inflammatory lesions are seen in the pancreas, and around the arteries and veins in the pancreas
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• lymphoid infiltrates consisting of small lymphoid cells, plasma cells, and large immunoblasts are found in the portal spaces of the liver
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• inflammatory lesions are seen in the interstitium and around arteries and veins in the cortex and medulla of the kidney
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• occurs in 60% of mice by 3 months of age
• is characterized by glomerular lesions and tubular cast formation
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• dense bronchiovascular lymphoid infiltrates consisting of small lymphoid cells, plasma cells, and large immunoblasts
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cardiovascular system
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• mice develop phlebitis, with vascular lesions in the pancreas of 10% and 11.1% of 10 and 20 week old mice and obliterating phlebitis in lungs of 70% and 77.8% of 10 and 20 week old mice, respectively
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homeostasis/metabolism
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• 80 ug/ml of urea detected in mice by 3 months of age
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albuminuria
(
J:125722
)
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• develops in 60% of mice by three months of age
• albumin levels are over 1mg/ml in some mice
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cellular
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• 2.2-fold increase in number of proliferating CD4-positive T cells isolated from spleen and lymph nodes
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digestive/alimentary system
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• mice develop massive fibrotic lesions in salivary glands
• fibrosis occurs in areas close to inflammatory lesions associated with the infiltration of lymphoplasmacytic cells
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• multiple inflammatory lesions are seen around the ducts in the salivary glands
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renal/urinary system
albuminuria
(
J:125722
)
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• develops in 60% of mice by three months of age
• albumin levels are over 1mg/ml in some mice
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• inflammatory lesions are seen in the interstitium and around arteries and veins in the cortex and medulla of the kidney
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• occurs in 60% of mice by 3 months of age
• is characterized by glomerular lesions and tubular cast formation
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• deposits of IgE in the are observed in glomerular capillary wall
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• mice develop massive fibrotic lesions in the kidneys
• fibrosis occurs in areas close to inflammatory lesions associated with the infiltration of lymphoplasmacytic cells
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growth/size/body
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• body weight becomes lower after 16 weeks of age
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• spleen and lymph nodes start to enlarge at 7 weeks of age
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• spleen weight becomes higher after 6 weeks of age
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• spleen cellularity is increased 5 times compared to in wild-type mice
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liver/biliary system
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• lymphoid infiltrates consisting of small lymphoid cells, plasma cells, and large immunoblasts are found in the portal spaces of the liver
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respiratory system
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• dense bronchiovascular lymphoid infiltrates consisting of small lymphoid cells, plasma cells, and large immunoblasts
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endocrine/exocrine glands
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• mice develop massive fibrotic lesions in salivary glands
• fibrosis occurs in areas close to inflammatory lesions associated with the infiltration of lymphoplasmacytic cells
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• multiple inflammatory lesions are seen around the ducts in the salivary glands
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• thymus cellularity is one tenth of wild-type
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• mice develop massive fibrotic lesions in the pancreas
• fibrosis occurs in areas close to inflammatory lesions associated with the infiltration of lymphoplasmacytic cells
• however, storiform fibrosis is not seen
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• multiple inflammatory lesions are seen in the pancreas, and around the arteries and veins in the pancreas
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hematopoietic system
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• 2.2-fold increase in number of proliferating CD4-positive T cells isolated from spleen and lymph nodes
|
|
• thymus cellularity is one tenth of wild-type
|
|
• spleen and lymph nodes start to enlarge at 7 weeks of age
|
|
• spleen weight becomes higher after 6 weeks of age
|
|
• spleen cellularity is increased 5 times compared to in wild-type mice
|
|
• is present in some mice by 3 months of age
|
|
• after 4 weeks, mice exhibit high levels of eosinophils
|
|
• severe but partial block in development of DN alpha-beta T cells into DP T cells
|
|
• the number of double positive cells decrease after birth and are almost undetectable by 7 weeks of age
|
|
• gamma-delta T cell development is unaffected in these mice
|
|
• at 6 weeks, the number of B cells in secondary lymphoid organs is increased 7 to 10 times compared to wild-type
• B cells in enlarged secondary lymphoid organs exhibit 25% hyperactivated phenotype, 50% antibody-producing phenotype and 25% a resting phenotype
|
|
• 500-fold increase in plasma cells in the spleen and lymph nodes of 6 week old mice
• 5% of total lymph node cells are plasma cells by 6 weeks of age
|
|
• the ratio of CD4 to CD8 cells is skewed towards CD4 cells
• the ratio of single positive T cells in the secondary lymphoid organs is bias towards CD4+ cells and this population expands over time
|
|
• the number of CD4+ cells increases due to increased proliferation and extended survival
(J:77098)
• are first observed in secondary lymph nodes at 4 weeks of age
(J:125722)
|
|
• during the first weeks of life, mice develop a Th2 lymphoproliferative disorder
|
|
• the majority of T cells found in the periphery have cell surface markers of memory T cells (low TCR expression, CD44-high, CD62L-low, CD69-positive)
|
|
• germinal centers form in every B cell follicle starting at 4 weeks of age
|
|
• are pale and have accumulations of plasma and immunoblast cells
|
|
• 25% of B cells from secondary lymphoid organs have cell surface markers indicative of a hyperactive state
• an additional 50% of B cells are activated compared to B cells from wild-type mice that almost exclusively in a resting state
|
|
• up to 10,000 times wild-type
(J:77098)
• adult mice have serum levels of IgE that are 10,000 times greater than normal
(J:125722)
• antibodies are polyclonal
(J:125722)
|
|
• up to 200 times wild-type
(J:77098)
• adult mice have serum levels that are 200 times greater than normal
(J:125722)
• antibodies are polyclonal
(J:125722)
• serum IgG1 levels remain elevated in mice treated with corticosteroid at 4 or 7-8 weeks of age
(J:262891)
|
|
• 6-fold greater levels in the sera of adult mice
|
|
• wild-type B cells secrete abnormally high amounts of IgG1 when cultured with mutant CD4 T cells
• this effect is dependent on IL-4
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| IgG4-related disease | DOID:0080356 | J:262891 | ||
