mortality/aging
• approximately 50% of homozygotes survive until 4 weeks of age
|
hematopoietic system
N |
• normal numbers of splenic IgM+ B220+ B cells
• splenocytes have normal expression of IL2RB, IL2RG, IL15RA and IL18
|
• increased numbers of splenic and lymph node plasma cells with serum IgM levels elevated by approximately 5 fold
|
• reduced NK cell numbers evidenced by diminished splenic DX5+ CD3- cells and diminished splenic NK1.1+ CD3- cells
• lymph nodes and bone marrow do not contain detectable DX5+ CD3- cells
|
• slightly reduced numbers of splenic T cells, particularly CD8+ T cells
|
• 4 to 8 week old homozygotes have approximately 65% reduction in thymocyte numbers with low levels of CD4 expressed on many CD8+ thymocytes
|
• poly (I:C) stimulated splenocytes produce reduced amounts of IFNG and IL12
|
• splenocytes fail to lyse YAC-1 tumor cells, B2m-/- lyphoid blasts, and MHC class I deficient RMA-S tumor cells are not rejected at a normal rate in vivo
• addition of IL12, IL2 and IL15 fails to restore YAC-1 cytolysis
|
• splenic T cells display a defect in activation following cross-linking of the T cell receptor
|
immune system
• increased numbers of splenic and lymph node plasma cells with serum IgM levels elevated by approximately 5 fold
|
• reduced NK cell numbers evidenced by diminished splenic DX5+ CD3- cells and diminished splenic NK1.1+ CD3- cells
• lymph nodes and bone marrow do not contain detectable DX5+ CD3- cells
|
• slightly reduced numbers of splenic T cells, particularly CD8+ T cells
|
• 4 to 8 week old homozygotes have approximately 65% reduction in thymocyte numbers with low levels of CD4 expressed on many CD8+ thymocytes
|
• poly (I:C) stimulated splenocytes produce reduced amounts of IFNG and IL12
|
• splenocytes fail to lyse YAC-1 tumor cells, B2m-/- lyphoid blasts, and MHC class I deficient RMA-S tumor cells are not rejected at a normal rate in vivo
• addition of IL12, IL2 and IL15 fails to restore YAC-1 cytolysis
|
• splenic T cells display a defect in activation following cross-linking of the T cell receptor
|
endocrine/exocrine glands
• 4 to 8 week old homozygotes have approximately 65% reduction in thymocyte numbers with low levels of CD4 expressed on many CD8+ thymocytes
|
cellular
• splenic T cells display a defect in activation following cross-linking of the T cell receptor
|