Analysis Tools
mortality/aging
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• 50% mortality is observed at 5 months with 90% mortality at 7.5 months, significantly reduced from wild-type
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cellular
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• mild increase in mesangial cells is seen at 20 weeks of age
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• larger lymph nodes often show extensive necrosis
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immune system
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• larger lymph nodes often show extensive hemorrhage
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• larger lymph nodes often show extensive necrosis
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• CD19+IgM+ immature B cells are reduced in the spleen
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• numbers of the T1 subset of B cells is reduced
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• mild to moderated neutrophil accumulation is observed at 20 weeks
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• CD19+ IgDhigh IgMlow B cells are severely reduced in the spleen
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• numbers of marginal zone (MZ) B cells is reduced
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• increased B220+IgM+ cells are observed in bone marrow; number of IgG-secreting cells are significantly increased compared to Faslpr homozygotes
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• staining intensity and number of germinal centers (GCs) is reduced 10 days post-challenge with NP-KLH antigen, compared to controls
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• in vitro, splenic B cells produce significantly higher amounts of IgG1 in response to LPS and anti-CD40 plus Il4 stimulation, and higher amounts of IgG2a upon LPS stimulation
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• production of anti-NP IgG is impaired in spleen cells
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• IgG and IgM levels are increased in serum at 6 months
(J:7454)
• mice display hypergammaglobulinemia; serum levels are comparable to Fas homozygotes
(J:122315)
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• mice exhibit increased serum IgG levels and IgG deposits in the kidney unlike wild-type mice
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• nodes are 62 times normal size
(J:7454)
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• mice exhibit increased serum levels of kappa-chain rheumatoid factor and DNA auto-antibodies, lymphadenopathy, glomerulonephritis, renal immunoglobulin deposits, proteinuria, and end organ disease compared to in wild-type mice
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• at 16 weeks, levels of anti-cardiolipin antibodies are significantly higher than in wild-type controls; levels are significantly higher at 8 weeks
(J:14151)
• mice exhibit increased serum levels of kappa-chain rheumatoid factor compared to in wild-type mice
(J:92084)
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• mice have significantly increased levels of anti-ssDNA antibodies
(J:7454)
• at 16 weeks, anti-DNA titers are significantly higher than in wild-type controls
(J:14151)
• DNA auto-antibodies are increased compared to in wild-type mice at 12 weeks
(J:92084)
• by 5-6 months of age, Fas-deficient mice have antinuclear antibody (ANA) levels comparable to >50% of C4b-deficient females (on Ighb haplotype homozygous background)
(J:111811)
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• antibodies are increased relative to controls and other mutant strains with Faslpr mutations
(J:7454)
• mice produce high titers of IgG1 and IgG2a anti-dsDNA antibodies, comparable to Fas homozygotes
(J:122315)
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• at 20 weeks, all mice show mononuclear infiltrates in the choroid plexus; at 10 weeks, all mice display monuclear infiltrates
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• Background Sensitivity: severe immune complex glomerulonephritis develops by 6 months
(J:7454)
• mice show deposition of IgG or C3 in kidneys and inflammation, similar to Fas homozygotes
(J:122315)
• kidney lesions have lower scores than those in double mutants at 20 weeks
(J:127199)
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muscle
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• myocardium neighboring the heart valves shows mononuclear infiltration of the vessels, but valves are normal
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hematopoietic system
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• CD19+IgM+ immature B cells are reduced in the spleen
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• numbers of the T1 subset of B cells is reduced
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• mild to moderated neutrophil accumulation is observed at 20 weeks
|
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• CD19+ IgDhigh IgMlow B cells are severely reduced in the spleen
|
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• numbers of marginal zone (MZ) B cells is reduced
|
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• increased B220+IgM+ cells are observed in bone marrow; number of IgG-secreting cells are significantly increased compared to Faslpr homozygotes
|
|
• staining intensity and number of germinal centers (GCs) is reduced 10 days post-challenge with NP-KLH antigen, compared to controls
|
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• in vitro, splenic B cells produce significantly higher amounts of IgG1 in response to LPS and anti-CD40 plus Il4 stimulation, and higher amounts of IgG2a upon LPS stimulation
|
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• production of anti-NP IgG is impaired in spleen cells
|
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• IgG and IgM levels are increased in serum at 6 months
(J:7454)
• mice display hypergammaglobulinemia; serum levels are comparable to Fas homozygotes
(J:122315)
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• mice exhibit increased serum IgG levels and IgG deposits in the kidney unlike wild-type mice
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renal/urinary system
albuminuria
(
J:122315
)
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• mice have excessive urinary albumin compared to wild-type (>10-fold) at 3-4 months
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• foot processes are only focally effaced
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• dilated capsules are observed in mice at 3-4 months
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• abnormalities due to severe glomerulonephritis
(J:7454)
• at 20 weeks, lesions show some neutrophil infiltration and hypercellularity
(J:127199)
• tuft necrosis, capsular proliferation and fibrosis are less common and less severe than observed in double mutants
(J:127199)
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• mild increase in mesangial cells is seen at 20 weeks of age
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• mild increase in mesangial matrix is seen at 20 weeks of age
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• glomerulonephritic changes such as hypercellularity, lobularity, dilated capsules and crescent formation or enlarged glomeruli are observed in mice at 3-4 months
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• Background Sensitivity: severe immune complex glomerulonephritis develops by 6 months
(J:7454)
• mice show deposition of IgG or C3 in kidneys and inflammation, similar to Fas homozygotes
(J:122315)
• kidney lesions have lower scores than those in double mutants at 20 weeks
(J:127199)
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• crescent formation is observed in mice at 3-4 months
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• enlarged glomeruli are observed in mice at 3-4 months
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• progressive decline in renal function is observed, during progression to end-stage renal disease
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behavior/neurological
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• mice show increased latency to locate hidden platform in water maze testing on days 2-5 of testing at 8 weeks of age; at 16 weeks in spatial bias testing, mutants spend less time and travel reduced distances in quadrant of platform's previous location compared to controls
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• equilibrium is significantly impaired in mice at 18-20 weeks, as measured by performance in rotarod tests
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vision/eye
| N |
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cardiovascular system
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• myocardium neighboring the heart valves shows mononuclear infiltration of the vessels, but valves are normal
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• larger lymph nodes often show extensive hemorrhage
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hearing/vestibular/ear
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• widened intercellular space in the stria vascularis
• slight degenerative changes in the stria vascularis of both the apical and basal turns
• the basement membrane of the capillaries in the stria vascularis was thickened
• the basal infolding of strial marginal cells
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• thinned intermediate cell layer
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• the ABR threshold f the 20-week-old mutant mice were significantly higher than those of the 4-week-old mutant mice and the 20-week-old wild-type BALB/c mice
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nervous system
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• at 20 weeks, all mice show mononuclear infiltrates in the choroid plexus; at 10 weeks, all mice display monuclear infiltrates
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homeostasis/metabolism
albuminuria
(
J:122315
)
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• mice have excessive urinary albumin compared to wild-type (>10-fold) at 3-4 months
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• Background Sensitivity: 7-8 week old mice show 2-3 fold induction of Dnase1l3 in macrophages and 4-5 fold induction in splenocytes over C57BL/6; levels in other strains like BXSB/MpJ and (NZB x NZW)F1 are similarly elevated compared to B6
• Background Sensitivity: mice show a dramatic defect (~8-fold decrease) in macrophage-secreted Dnase1l3 barrier to liposomal (BT) activity compared to B6; (NZB x NZW)F1 mice show a similar defect in BT activity
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pigmentation
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• thinned intermediate cell layer
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digestive/alimentary system
endocrine/exocrine glands
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Sjogren's syndrome | DOID:12894 |
OMIM:270150 |
J:123192 | |
