cellular
• more apoptotic cells are detected relative to wild-type Tnfrsf9, Fas lpr controls
|
mortality/aging
• by 5 months, 80% mortality is observed compared to 40% in Tnfrsf9-sufficient, Fas-null mice; by 4 months, mice become increasingly moribund and display reduced activity
|
hematopoietic system
N |
• CD8beta+ T cells are not altered in number at any age
|
• significant enlargement of axillary lymph nodes is seen at 5 months compared to Tnfrsf9-wt, Fas-null mice
|
• in lacrimal glands, DN T cell percentages are about 2-fold greater than in wild-type Tnfrsf9, Fas lpr controls; similar increases are seen in spleen and salivary glands
(J:126929)
• increased proportion seen in spleen compared to Tnfrsf9-sufficient, Fas-null mice
(J:127197)
|
• percentages of B-1 (B220+ CD5+) cells are increased significantly in peritoneums and slightly higher in spleens
|
• percentages of B-2( B220+ CD5-) cells are increased significantly in peritoneums and slightly higher in spleens
|
• 3- and 5-month old mice show elevated CD4+ T cell numbers in lacrimal glands
(J:126929)
• mice display early increases in CD4+ T cells in spleen, and numbers keep rising
(J:127197)
|
• greatly increased germinal center formation is observed compared to Tnfrsf9-sufficient, Fas-null mice
|
• circulating titres are elevated compared with compared to Tnfrsf9-sufficient, Fas-null mice
|
immune system
• extensive and early inflammation is observed compared with control wild-type Tnfrsf9, Fas lpr homozygotes
• by 3 months, extensive mononuclear cell infiltration is observed, becoming intense by 5 months while controls show no infiltrate at 1 month, mimimal inflammatory lesions at 3 months and extensive, although less severe than double mutants, infiltrates by 5 months of age
|
• significant enlargement of axillary lymph nodes is seen at 5 months compared to Tnfrsf9-wt, Fas-null mice
|
• in lacrimal glands, DN T cell percentages are about 2-fold greater than in wild-type Tnfrsf9, Fas lpr controls; similar increases are seen in spleen and salivary glands
(J:126929)
• increased proportion seen in spleen compared to Tnfrsf9-sufficient, Fas-null mice
(J:127197)
|
• percentages of B-1 (B220+ CD5+) cells are increased significantly in peritoneums and slightly higher in spleens
|
• percentages of B-2( B220+ CD5-) cells are increased significantly in peritoneums and slightly higher in spleens
|
• 3- and 5-month old mice show elevated CD4+ T cell numbers in lacrimal glands
(J:126929)
• mice display early increases in CD4+ T cells in spleen, and numbers keep rising
(J:127197)
|
• greatly increased germinal center formation is observed compared to Tnfrsf9-sufficient, Fas-null mice
|
• circulating titres are elevated compared with compared to Tnfrsf9-sufficient, Fas-null mice
|
• significant enlargement at 5 months compared to Tnfrsf9-wt, Fas-null mice
|
• in lacrimal glands, increased levels of Il-4 are detected compared to controls
|
• phenotypic manifestations are increased relative to Faslpr homozygotes
|
• serum titres of anti-nuclear IgG1/2a are increased at 3 and 5 months
• anti-DNA antibody production is increased compared to Tnfrsf9-sufficient, Fas-null mice
|
renal/urinary system
• increased immunoglobulin deposits are detected in kidneys compared to controls
|
• mice show exacerbated renal injury, with increased glomerular infiltrates
|
• IgG and C3 depositions in kidneys are increased compared with Tnfrsf9-sufficient, Fas-null mice
|
craniofacial
• from 4 months of age, >60% of mice display progressive erosion of pinnae
|
hearing/vestibular/ear
• from 4 months of age, >60% of mice display progressive erosion of pinnae
|
endocrine/exocrine glands
• more apoptotic cells are detected relative to wild-type Tnfrsf9, Fas lpr controls
|
• increased deposits of IgG1, but not IgG2a are detected in lacrimal glands at 3 and 5 months
|
• extensive and early inflammation is observed compared with control wild-type Tnfrsf9, Fas lpr homozygotes
• by 3 months, extensive mononuclear cell infiltration is observed, becoming intense by 5 months while controls show no infiltrate at 1 month, mimimal inflammatory lesions at 3 months and extensive, although less severe than double mutants, infiltrates by 5 months of age
|
vision/eye
• more apoptotic cells are detected relative to wild-type Tnfrsf9, Fas lpr controls
|
• increased deposits of IgG1, but not IgG2a are detected in lacrimal glands at 3 and 5 months
|
• extensive and early inflammation is observed compared with control wild-type Tnfrsf9, Fas lpr homozygotes
• by 3 months, extensive mononuclear cell infiltration is observed, becoming intense by 5 months while controls show no infiltrate at 1 month, mimimal inflammatory lesions at 3 months and extensive, although less severe than double mutants, infiltrates by 5 months of age
|
integument
skin lesions
(
J:127197
)
• by 5 months, skin lesions around tip of nose and around ears are more pronounced than in Tnfrsf9-wt, Fas-null mice
|
growth/size/body
• from 4 months of age, >60% of mice display progressive erosion of pinnae
|
• significant enlargement of axillary lymph nodes is seen at 5 months compared to Tnfrsf9-wt, Fas-null mice
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Sjogren's syndrome | DOID:12894 |
OMIM:270150 |
J:120559 | |
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:120559 |