Mouse Genome Informatics
cx
    Faslpr/Faslpr
Tnfrsf9tm1Byk/Tnfrsf9tm1Byk

MRL.Cg-Tnfrsf9tm1Byk Faslpr
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
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 (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)
• 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)
• significant enlargement of axillary lymph nodes is seen at 5 months compared to Tnfrsf9-wt, Fas-null mice
• 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
• 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)
• 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)
• significant enlargement of axillary lymph nodes is seen at 5 months compared to Tnfrsf9-wt, Fas-null mice
• 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
• by 5 months, skin lesions around tip of nose and around ears are more pronounced than in Tnfrsf9-wt, Fas-null mice

Mouse Models of Human Disease
OMIM IDRef(s)
Sjogren Syndrome 270150 J:120559
Systemic Lupus Erythematosus; SLE 152700 J:120559