Mouse Genome Informatics
tg
    Tg(Psp-Rbbp4)1Yoha/0
involves: C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
immune system
• mice exhibit an increase in CD21high IgMhigh B220+ marginal zone B cells in the cervical lymph nodes and spleen compared to in wild-type mice
• markers of T cell activation are up-regulated on CD4+ T cells in the cervical lymph nodes compared to in wild-type mice
• unlike in wild-type mice, salivary gland epithelial cells function as antigen presenting cells
• anti-TCR-beta and CD28 antibody stimulated cervical lymph node T cells exhibit higher levels of IFN-gamma compared to similarly treated wild-type cells
• IFN-gamma production in salivary and lacrimal gland epithelia is increased compared to in wild-type mice
• IL18 production in salivary gland epithelia is increased compared to in wild-type mice
• anti-TCR-beta and CD28 antibody stimulated cervical lymph node T cells exhibit higher levels of IL2 compared to similarly treated wild-type cells
• at 24 weeks of age, mice exhibit autoimmune exocrinopathy resembling Sjogren Syndrome
• autoimmune exocrinopathy is transferable by adoptive transfer of cervical lymph node cells and is more severe in Rag2 null mice that have been ovariectomized
• mice exhibit an increase autoantibodies against SS-A(Ro), SS-B(La) and alpha-fodrin compared to in wild-type mice

endocrine/exocrine glands
• by 30 to 50 weeks of age, lymphocyte infiltration is present in the salivary gland unlike in wild-type mice with higher incidences of inflammatory lesions observed in female mice
• inflammatory infiltrates are composed of mainly Thy1.2+ CD4+ T cells with a minor proportion of B220+ B cells, CD8+ T cells and CD11b+ cells
• unlike in wild-type mice, salivary gland epithelial cells function as antigen presenting cells
• IFN-gamma and IL18 production in salivary gland epithelia is increased compared to in wild-type mice
• at 30 weeks of age or more
• by 30 to 50 weeks of age, lymphocyte infiltration is present in the lacrimal gland unlike in wild-type mice with higher incidences of inflammatory lesions observed in female mice
• inflammatory infiltrates are composed of mainly Thy1.2+ CD4+ T cells with a minor proportion of B220+ B cells, CD8+ T cells and CD11b+ cellsinflammatory infiltrates are composed of mainly Thy1.2+ CD4+ T cells with a minor proportion of B220+ B cells, CD8+ T cells and CD11b+ cells
• at 30 weeks of age or more, tear volume is reduced compared to in wild-type mice
• lacrimal gland expression of IFN-gamma is increased compared to in wild-type mice

vision/eye
• by 30 to 50 weeks of age, lymphocyte infiltration is present in the lacrimal gland unlike in wild-type mice with higher incidences of inflammatory lesions observed in female mice
• inflammatory infiltrates are composed of mainly Thy1.2+ CD4+ T cells with a minor proportion of B220+ B cells, CD8+ T cells and CD11b+ cellsinflammatory infiltrates are composed of mainly Thy1.2+ CD4+ T cells with a minor proportion of B220+ B cells, CD8+ T cells and CD11b+ cells
• at 30 weeks of age or more, tear volume is reduced compared to in wild-type mice
• lacrimal gland expression of IFN-gamma is increased compared to in wild-type mice

digestive/alimentary system
• by 30 to 50 weeks of age, lymphocyte infiltration is present in the salivary gland unlike in wild-type mice with higher incidences of inflammatory lesions observed in female mice
• inflammatory infiltrates are composed of mainly Thy1.2+ CD4+ T cells with a minor proportion of B220+ B cells, CD8+ T cells and CD11b+ cells
• unlike in wild-type mice, salivary gland epithelial cells function as antigen presenting cells
• IFN-gamma and IL18 production in salivary gland epithelia is increased compared to in wild-type mice
• at 30 weeks of age or more

hematopoietic system
• mice exhibit an increase in CD21high IgMhigh B220+ marginal zone B cells in the cervical lymph nodes and spleen compared to in wild-type mice
• markers of T cell activation are up-regulated on CD4+ T cells in the cervical lymph nodes compared to in wild-type mice

homeostasis/metabolism
• at 30 weeks of age or more

Mouse Models of Human Disease
OMIM IDRef(s)
Sjogren Syndrome 270150 J:141378