Phenotypes Associated with This Genotype

Genotype
MGI:4829879

• Allelic Composition: Nfkbia^tm1.1Pjc/Nfkbia^tm1.1Pjc
• Genetic Background: involves: C57BL/6

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

mortality/aging

increased susceptibility to bacterial infection induced morbidity/mortality ( J:163686 )

• hypersensitive to LPS induced lethality

premature death ( J:163686 )

• become sick and die at about 13 - 15 months of age

immune system

increased T cell apoptosis ( J:163686 )

• T cells are hyperresponsive to activation induced cell death

abnormal T cell number ( J:163686 )

increased thymocyte number ( J:163686 )

increased double-positive T cell number ( J:163686 )

decreased CD4-positive, alpha-beta T cell number ( J:163686 )

• decreased in both percentage and absolute number

decreased CD8-positive, alpha-beta T cell number ( J:163686 )

• decreased in both percentage and absolute number

increased memory T cell number ( J:163686 )

• T cells derived from the lungs and peripheral lymph organs are predominantly memory T cells rather than naive T cells

increased activated T cell number ( J:163686 )

• at 6 weeks of age activated T cell numbers are increases in the lungs, lymph nodes, and spleen

abnormal T cell activation ( J:163686 )

• T cells are hyperresponsive to in vitro activation and activation induced cell death

• splenic T cells produce more IL2 and IFNG when stimulated with a low dose of anti-CD3/CD28 antibodies

increased T cell proliferation ( J:163686 )

• in splenic T cells in vitro when stimulated with a low dose of anti-CD3/CD28 antibodies

abnormal circulating cytokine level ( J:163686 )

• hypersensitive to LPS induced proinflammatory cytokine production

increased circulating interleukin-1 alpha level ( J:163686 )

• at about 13 - 15 months of age

increased circulating interleukin-17 level ( J:163686 )

• at about 13 - 15 months of age

increased circulating tumor necrosis factor level ( J:163686 )

• at about 13 - 15 months of age

autoimmune response ( J:163686 )

increased anti-insulin autoantibody level ( J:163686 )

• at 5 to 7 months of age

increased anti-nuclear antigen antibody level ( J:163686 )

• increase in anti-DNA, anti-Ro/SS-A, and anti-La/SS-B antibodies at 5 to 7 months of age

chronic inflammation ( J:163686 )

• extensive perivascular lymphocytic infiltration is seen in the pancreas, liver, lacrimal glands, salivary glands, and lungs of 3 month old mice

increased susceptibility to bacterial infection induced morbidity/mortality ( J:163686 )

• hypersensitive to LPS induced lethality

homeostasis/metabolism

abnormal circulating cytokine level ( J:163686 )

• hypersensitive to LPS induced proinflammatory cytokine production

increased circulating interleukin-1 alpha level ( J:163686 )

• at about 13 - 15 months of age

increased circulating interleukin-17 level ( J:163686 )

• at about 13 - 15 months of age

increased circulating tumor necrosis factor level ( J:163686 )

• at about 13 - 15 months of age

hematopoietic system

increased T cell apoptosis ( J:163686 )

• T cells are hyperresponsive to activation induced cell death

abnormal T cell number ( J:163686 )

increased thymocyte number ( J:163686 )

increased double-positive T cell number ( J:163686 )

decreased CD4-positive, alpha-beta T cell number ( J:163686 )

• decreased in both percentage and absolute number

decreased CD8-positive, alpha-beta T cell number ( J:163686 )

• decreased in both percentage and absolute number

increased memory T cell number ( J:163686 )

• T cells derived from the lungs and peripheral lymph organs are predominantly memory T cells rather than naive T cells

increased activated T cell number ( J:163686 )

• at 6 weeks of age activated T cell numbers are increases in the lungs, lymph nodes, and spleen

abnormal T cell activation ( J:163686 )

• T cells are hyperresponsive to in vitro activation and activation induced cell death

• splenic T cells produce more IL2 and IFNG when stimulated with a low dose of anti-CD3/CD28 antibodies

increased T cell proliferation ( J:163686 )

• in splenic T cells in vitro when stimulated with a low dose of anti-CD3/CD28 antibodies

cellular

increased T cell apoptosis ( J:163686 )

• T cells are hyperresponsive to activation induced cell death

increased T cell proliferation ( J:163686 )

• in splenic T cells in vitro when stimulated with a low dose of anti-CD3/CD28 antibodies

endocrine/exocrine glands

increased thymocyte number ( J:163686 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Sjogren's syndrome		DOID:12894	OMIM:270150	J:163686