Phenotypes Associated with This Genotype

Genotype
MGI:3822909

• Allelic Composition: Nfkb1^tm1Sley/Nfkb1^tm1Sley
• Genetic Background: involves: 129S4/SvJae * 129S8/SvEv

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

immune system

decreased T cell proliferation ( J:142340 )

• nave CD4⁺ T cells stimulated in vitro through the TCR only have a third the proliferative response as control mice

• 66% fewer naive CD4⁺ T cells go into S-phase with 24 hours of TCR stimulation compared to controls

• the S-phase entry defect can be partly overcome by CD28 stimulation

• allogeneic proliferative response of nave CD4⁺ T cells is reduced 6-fold compared to controls

• the amount of IL-2 required for a half maximal proliferation response is 100-fold higher than in controls

decreased memory T cell number ( J:142340 )

• CD4⁺ memory T cells are reduced by 3-fold in the spleen

• splenic CD8⁺ memory T cells are also reduced

• reconstitution of wild-type mice with both wild-type and mutant bone marrow demonstrate an intrinsic defect of mutant hematopoietic cells to produce CD4⁺ memory T cells

decreased regulatory T cell number ( J:142340 )

• there is a 75% decrease in the number of regulatory T cells found in the spleen, lymph node and thymus of mice

• reconstitution of wild-type mice with both wild-type and mutant bone marrow demonstrate an intrinsic defect of mutant hematopoietic cells to produce CD4⁺ regulatory T cells

decreased circulating interleukin-2 level ( J:142340 )

• nave CD4⁺ T cells only produce one-third the amount of IL-2 as controls after in vitro stimulation

abnormal inguinal lymph node morphology ( J:142340 )

• most mice have small or absent inguinal lymph nodes

absent inguinal lymph nodes ( J:142340 )

small inguinal lymph nodes ( J:142340 )

hematopoietic system

decreased T cell proliferation ( J:142340 )

• nave CD4⁺ T cells stimulated in vitro through the TCR only have a third the proliferative response as control mice

• 66% fewer naive CD4⁺ T cells go into S-phase with 24 hours of TCR stimulation compared to controls

• the S-phase entry defect can be partly overcome by CD28 stimulation

• allogeneic proliferative response of nave CD4⁺ T cells is reduced 6-fold compared to controls

• the amount of IL-2 required for a half maximal proliferation response is 100-fold higher than in controls

decreased memory T cell number ( J:142340 )

• CD4⁺ memory T cells are reduced by 3-fold in the spleen

• splenic CD8⁺ memory T cells are also reduced

• reconstitution of wild-type mice with both wild-type and mutant bone marrow demonstrate an intrinsic defect of mutant hematopoietic cells to produce CD4⁺ memory T cells

decreased regulatory T cell number ( J:142340 )

• there is a 75% decrease in the number of regulatory T cells found in the spleen, lymph node and thymus of mice

• reconstitution of wild-type mice with both wild-type and mutant bone marrow demonstrate an intrinsic defect of mutant hematopoietic cells to produce CD4⁺ regulatory T cells

homeostasis/metabolism

decreased circulating interleukin-2 level ( J:142340 )

• nave CD4⁺ T cells only produce one-third the amount of IL-2 as controls after in vitro stimulation

cellular

decreased T cell proliferation ( J:142340 )

• nave CD4⁺ T cells stimulated in vitro through the TCR only have a third the proliferative response as control mice

• 66% fewer naive CD4⁺ T cells go into S-phase with 24 hours of TCR stimulation compared to controls

• the S-phase entry defect can be partly overcome by CD28 stimulation

• allogeneic proliferative response of nave CD4⁺ T cells is reduced 6-fold compared to controls

• the amount of IL-2 required for a half maximal proliferation response is 100-fold higher than in controls