Analysis Tools
mortality/aging
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• mice die prematurely from multiple pathologies starting as early as 15 weeks of age
• 43% of mutant mice have died by 50 weeks of age compared to none in the wild-type control group
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immune system
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• granulopoiesis is increased in the bone marrow
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• increased plasmacytoid cells are found in the lymph nodes
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• B cell numbers are increased in older mice 2- to 8- fold
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• T cell numbers are increased in older mice 2- to 8- fold
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• B cells expressing activation markers are increased 26% in wild-type to 62% and 70%, in mild and severe lymphadenopathy, respectively
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• mean IgA levels are 35-fold higher than controls by 14 weeks of age
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• there is a 12-fold increase in IgG2a levels over controls in 14 week old mice
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• T cells expressing activation markers are increased from 21% in wild-type to 34% in mice with mild lymphadenopathy, and 57% in mice with severe lymphadenopathy
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• spleens of mice over 15 weeks of age weigh 3-10 times as much as controls
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• expansion of the red pulp is noted in mice over 15 weeks of age
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• expansion of the white pulp is noted in mice over 15 weeks of age
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• 70% of mice over 15 weeks of age develop lymphadenopathy with about half the cases being severe
• generalized lymphadenopathy is observed in severe cases while lymphadenopathy is often confined to the cervical and submandibular lymph nodes in mild cases
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• lymph node cell numbers are 2- to 8- fold greater than controls in older mice with swollen lymph nodes
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• autoimmune lupus nephritis with autoantibody production leading to renal failure
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• 63% of homozygous mice have anti-dsDNA antibodies
• auto-antibodies are detected as earlier as 9 weeks of age
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• kidneys have interstitial nephritis characterized by the presence of numerous mononuclear cells, tubular epithelial cell injury, interstitial fibrosis, and edema
• the glomeruli exhibited increase cellularity, thickened capillary loops, and an increase in mesangial matrix
• karyorrhexis was noted in a segmental pattern
• numerous subendothelial and mesangial deposits of immunoglobulin proteins are visible, along with widespread epithelial cell foot process effacement
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renal/urinary system
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• proteinura greater than 30 mg/dl is observed at 6 weeks of age
• by 22 weeks of age, 54% of mice exhibit abnormal proteinura
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• kidneys have interstitial nephritis characterized by the presence of numerous mononuclear cells, tubular epithelial cell injury, interstitial fibrosis, and edema
• the glomeruli exhibited increase cellularity, thickened capillary loops, and an increase in mesangial matrix
• karyorrhexis was noted in a segmental pattern
• numerous subendothelial and mesangial deposits of immunoglobulin proteins are visible, along with widespread epithelial cell foot process effacement
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• some mice suffer from oliguria shortly before dying suggesting kidney failure
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digestive/alimentary system
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• some female mice with kidney problems have extended stomachs full of undigested food
• these mice appear extremely thin and lethargic
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hematopoietic system
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• granulopoiesis is increased in the bone marrow
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• spleens of mice over 15 weeks of age weigh 3-10 times as much as controls
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• erythropoiesis in the bone marrow is decreased
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• along with numerous megakaryocytes, islands of erythropoiesis and granulopoiesis are observed throughout the enlarged spleens
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• increased plasmacytoid cells are found in the lymph nodes
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• B cell numbers are increased in older mice 2- to 8- fold
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• T cell numbers are increased in older mice 2- to 8- fold
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• expansion of the red pulp is noted in mice over 15 weeks of age
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• expansion of the white pulp is noted in mice over 15 weeks of age
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• B cells expressing activation markers are increased 26% in wild-type to 62% and 70%, in mild and severe lymphadenopathy, respectively
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• mean IgA levels are 35-fold higher than controls by 14 weeks of age
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• there is a 12-fold increase in IgG2a levels over controls in 14 week old mice
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• T cells expressing activation markers are increased from 21% in wild-type to 34% in mice with mild lymphadenopathy, and 57% in mice with severe lymphadenopathy
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homeostasis/metabolism
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• proteinura greater than 30 mg/dl is observed at 6 weeks of age
• by 22 weeks of age, 54% of mice exhibit abnormal proteinura
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cellular
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• granulopoiesis is increased in the bone marrow
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growth/size/body
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• spleens of mice over 15 weeks of age weigh 3-10 times as much as controls
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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:66501 | |
