Mouse Genome Informatics
tg1
    Tg(ACTB-TNFRSF6B)754Jwu/?
B6.Cg-Tg(Actb-TNFRSF6B)754Jwu
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• shortened lifespan is observed mostly in females and transgenic mice with lymphadenopathy
• survival rate for 14 month old females is 63.2%
• survival rate for 14 month old males is 81.8%

immune system
• leukocytopenia occurs in 6/10 female transgenic mice older than 6 months of age
• peripheral blood from transgenic mice older than 6 months of age contains a increased percentage of CD4+ T cell exhibiting CD62Llow and CD44high
• occurs with lymphadenopathy
• observed in 9 month old mice
• transgenic mice exhibit higher concentrations of IgE between 2 and 6 months
• lymphadenopathy is observed in 6-8 month old mice
• penetrance is higher in females (64.5%) than males (20%)
• usually accompanied by splenomegaly
• delayed type hypersensitivity reaction is compromised in transgenic mice
• autoantibodies found against mutiple self tissues (liver, lung, kidney, brain) in 7 month old female with lymphadenopathy
• 77% of female and 55% of male transgenic mice are anti-dsDNA antibody positive

hematopoietic system
• observed in female transgenic mice older than 6 months of age
• leukocytopenia occurs in 6/10 female transgenic mice older than 6 months of age
• observed in 3/9 female transgenic mice older than 6 months of age
• peripheral blood from transgenic mice older than 6 months of age contains a increased percentage of CD4+ T cell exhibiting CD62Llow and CD44high
• occurs with lymphadenopathy
• observed in 9 month old mice
• transgenic mice exhibit higher concentrations of IgE between 2 and 6 months

homeostasis/metabolism
• mild edema and vasodilation of capillaries and small vessels observed in dermis

liver/biliary system
• mice older than 6 months of age exhibit periarterial lymphocyte infiltration

renal/urinary system
• IgG and C3 deposition is observed in glomeruli and interstitial spaces
• mesangial hypertrophy
• mice older than 6 months of age exhibit enlarged glomeruli with increased glomerular epithelial cellularity and mesangial hypertrophy
• mice older than 6 months of age exhibit renal interstitial fibrosis

integument
• mild edema and vasodilation of capillaries and small vessels observed in dermis
• area under lesions contains granulomatous tissue consisting of collagen fibers, fibroblasts and infiltration lymphocytes
• mild edema and vasodilation of capillaries and small vessels observed in dermis
• hyperkeratosis and polymorphonuclear cell infiltration with follicular plugging is found in epidermal lesions
• junction between the epidermis and dermis in unclear
• 40% of 12+ month old females develop lesions
• lesions are characterized by superficial crusting and erosion

Mouse Models of Human Disease
OMIM IDRef(s)
Systemic Lupus Erythematosus; SLE 152700 J:148162