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Phenotypes Associated with This Genotype
Genotype
MGI:7281769
Allelic
Composition
Satb1tm2Kos/Satb1tm2Kos
Commd10Tg(Vav1-icre)A2Kio/Commd10+
Genetic
Background
involves: C57BL/6 * C57BL/10 * CBA/Ca
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Commd10Tg(Vav1-icre)A2Kio mutation (3 available); any Commd10 mutation (24 available)
Satb1tm2Kos mutation (0 available); any Satb1 mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice start to die around 24 weeks of age
• renal failure is cause of death

endocrine/exocrine glands
• autoimmune destruction of the salivary gland
• saliva production is decreased by 4 weeks of age in females and decreased in both males and females by 8 weeks of age; saliva production reaches the lowest level at around 8 weeks
• immune cell infiltration and destruction of the salivary gland is seen at 4 weeks of age and continues even after 8 weeks of age; magnitude of immune cell infiltration progressively increases with age
• CD4+ T cells are the major cell infiltrates at 4 weeks, however CD8+ T cells and CD4+CD8+ cells are present
• number of B cells gradually increase and dominate in older mice
• immune cell infiltration is seen in the pancreas, however impaired glucose tolerance is not seen

immune system
• immune cell infiltration and destruction of the salivary gland is seen at 4 weeks of age and continues even after 8 weeks of age; magnitude of immune cell infiltration progressively increases with age
• CD4+ T cells are the major cell infiltrates at 4 weeks, however CD8+ T cells and CD4+CD8+ cells are present
• number of B cells gradually increase and dominate in older mice
• immune cell infiltration is seen in the pancreas, however impaired glucose tolerance is not seen
• splenic Foxp3+CD25+ Treg cells are absent in mice from birth until 1 week of age, however no difference in the number of Treg cells is seen after 2 weeks of age
• mice develop Sjogren's syndrome by 4 weeks of age, with females being more susceptible and presenting earlier onset of the disease than males
• mice show T cell-dominant immune cell infiltration in the salivary glands at 4 weeks and a gradual increase in the frequency of B cells, and an increase in anti-SSA and anti-SSB antibodies around 8 weeks of age
• transfer or T cells from mutant cervical lymph nodes, but not spleen, is sufficient to induce the development of Sjogren's syndrome in RAG2 null mice
• 3-day-old mice transferred with Treg cells derived from mature wild-type spleen show improved xerostomia, although saliva production is still reduced, a lesser degree of lymphocyte infiltration into salivary glands is seen and mice are protected against development of Sjogren's syndrome
• mice develop lupus nephritis
• presence of anti-SSA and anti-SSB antibodies which are higher than in wild-type mice at 10 and 7 weeks of age, respectively
• ANA are detected at high levels after 15 weeks of age
• mice develop lupus nephritis after failure of salivary gland function

digestive/alimentary system
• autoimmune destruction of the salivary gland
• saliva production is decreased by 4 weeks of age in females and decreased in both males and females by 8 weeks of age; saliva production reaches the lowest level at around 8 weeks
• immune cell infiltration and destruction of the salivary gland is seen at 4 weeks of age and continues even after 8 weeks of age; magnitude of immune cell infiltration progressively increases with age
• CD4+ T cells are the major cell infiltrates at 4 weeks, however CD8+ T cells and CD4+CD8+ cells are present
• number of B cells gradually increase and dominate in older mice

hematopoietic system
• splenic Foxp3+CD25+ Treg cells are absent in mice from birth until 1 week of age, however no difference in the number of Treg cells is seen after 2 weeks of age

homeostasis/metabolism
• saliva production is decreased by 4 weeks of age in females and decreased in both males and females by 8 weeks of age; saliva production reaches the lowest level at around 8 weeks
• some mice exhibit proteinuria after 15 weeks of age

renal/urinary system
• some mice exhibit proteinuria after 15 weeks of age
• mice develop lupus nephritis after failure of salivary gland function
• IgM and IgG deposition, most likely a part of immune complexes, is seen around the glomerulus in the kidneys at 15 weeks of age
• a component of complement C3 is present in association with Ig deposition, suggestions that kidney lesions resembling lupus nephritis develop in aged mice
• no kidney lesions are seen at 4-5 weeks of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Sjogren's syndrome DOID:12894 OMIM:270150
J:252606


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
04/09/2024
MGI 6.23
The Jackson Laboratory