Phenotypes Associated with This Genotype

Genotype
MGI:3818860

• Allelic Composition: Tg(Psp-Rbbp4)1Yoha/0
• Genetic Background: involves: C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

immune system

salivary gland inflammation ( J:141378 )

• 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

lacrimal gland inflammation ( J:141378 )

• 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+ cells

increased marginal zone B cell number ( J:141378 )

• mice exhibit an increase in CD21^high IgM^high B220+ marginal zone B cells in the cervical lymph nodes and spleen compared to in wild-type mice

abnormal T cell activation ( J:141378 )

• markers of T cell activation are up-regulated on CD4+ T cells in the cervical lymph nodes compared to in wild-type mice

abnormal antigen presentation ( J:141378 )

• unlike in wild-type mice, salivary gland epithelial cells function as antigen presenting cells

increased interferon-gamma secretion ( J:141378 )

• 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

increased interleukin-18 secretion ( J:141378 )

• IL18 production in salivary gland epithelia is increased compared to in wild-type mice

increased interleukin-2 secretion ( J:141378 )

• anti-TCR-beta and CD28 antibody stimulated cervical lymph node T cells exhibit higher levels of IL2 compared to similarly treated wild-type cells

increased susceptibility to autoimmune disorder ( J:141378 )

• 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

increased autoantibody level ( J:141378 )

• mice exhibit an increase autoantibodies against SS-A(Ro), SS-B(La) and alpha-fodrin compared to in wild-type mice

endocrine/exocrine glands

abnormal salivary gland physiology ( J:141378 )

• 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

decreased salivation ( J:141378 )

• at 30 weeks of age or more

salivary gland inflammation ( J:141378 )

• 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

abnormal lacrimal gland physiology ( J:141378 )

• lacrimal gland expression of IFN-gamma is increased compared to in wild-type mice

lacrimal gland inflammation ( J:141378 )

• 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+ cells

vision/eye

abnormal lacrimal gland physiology ( J:141378 )

• lacrimal gland expression of IFN-gamma is increased compared to in wild-type mice

lacrimal gland inflammation ( J:141378 )

• 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+ cells

decreased tear production ( J:141378 )

• at 30 weeks of age or more, tear volume is reduced compared to in wild-type mice

digestive/alimentary system

abnormal salivary gland physiology ( J:141378 )

• 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

decreased salivation ( J:141378 )

• at 30 weeks of age or more

salivary gland inflammation ( J:141378 )

• 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

hematopoietic system

increased marginal zone B cell number ( J:141378 )

• mice exhibit an increase in CD21^high IgM^high B220+ marginal zone B cells in the cervical lymph nodes and spleen compared to in wild-type mice

abnormal T cell activation ( J:141378 )

• 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

decreased salivation ( J:141378 )

• at 30 weeks of age or more

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Sjogren's syndrome		DOID:12894	OMIM:270150	J:141378