immune system
• at 10 months of age, spleens of C4b-deficient females are enlarged with an average weight of 368 mg compared to controls which are ~100mg; most females have spleens 2-fold larger by weight, and 1/3 have spleens 4 times the average control weight
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• numbers are increased 4-fold over controls
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• numbers are increased 2-fold over controls
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• spontaneous germinal center formation is observed in C4b-deficient mice
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• clearance of soluble immune complexes (IC) injected into mice is delayed initially in C4b-deficient mice, resulting in a 2- to 3-fold increase in circulating IC levels compared to C2-deficient or B6.129-Ighb controls
• levels remain high for at least 70 minutes
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• by 5-6 months of age, >50% of C4b-deficient females (on Ighb haplotype homozygous background) have antinuclear antibody (ANA) levels comparable to Fas-deficient mice
• males have ANA levels higher (but not significant) than controls
• in 10-month old females, ANA levels as high as 1:30000 are present
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• IgG autoantibody titers against dsDNA are increased over controls in females at 10 months of age
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• IgG autoantibody titers against ss-DNA are increased over controls in females at 10 months of age
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• ~50% of 10-month old female C4b-deficient mice show glomerulonephritis but no male do
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renal/urinary system
• marked enlargement with hypercellularity is observed in the glomeruli of females at 10 months
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• ~50% of 10-month old female C4b-deficient mice show glomerulonephritis but no male do
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• many female mice display mesangial deposition of immune complexes (ICs) of IgG and C3
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hematopoietic system
• at 10 months of age, spleens of C4b-deficient females are enlarged with an average weight of 368 mg compared to controls which are ~100mg; most females have spleens 2-fold larger by weight, and 1/3 have spleens 4 times the average control weight
|
• numbers are increased 4-fold over controls
|
• numbers are increased 2-fold over controls
|
• spontaneous germinal center formation is observed in C4b-deficient mice
|
growth/size/body
• at 10 months of age, spleens of C4b-deficient females are enlarged with an average weight of 368 mg compared to controls which are ~100mg; most females have spleens 2-fold larger by weight, and 1/3 have spleens 4 times the average control weight
|
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:111811 |