immune system
|
• splenic T cells do not proliferate in response to Con A
|
|
• 22-fold reduction in cellularity
(J:22521)
• 10-fold less cellularity of thymus from fetal day E15.5 through birth
(J:111821)
• 20-fold less cellularity by six days of age
(J:111821)
|
|
• thymocytes do not activate in response to Con A
|
|
• in vitro class switch using LPS and IL-4 fail to generate more IgG1-positive cells
|
|
• complete absence of gamma-delta T cells in adult thymus
• few gamma-delta T cells found in fetal thymus
• TCR-gamma rearrangements are severely reduced
|
|
• 12-fold reduction in the spleen in 3-4 week old mice
• further loss occurs with age
|
|
• plasma cells in the gut are exceptionally rare
|
|
• 20-fold decrease in bone marrow though some cells are still present
|
|
• NK cells are not detected in the spleen
|
|
• 3 fold reduction of mature alphabeta T cells in the spleen
• reduction of T cells in lamina propia of gut is observed
|
|
• no gamma-delta T cells are found in peripheral lymphnoid organs or skin
|
|
• 12-fold reduction in number of T cells found in gut epithelium
|
|
• 8-fold reduction in cellularity
|
|
• no germinal centers are observed
|
|
• clusters of white pulp are diminished in number and size
|
|
• no IgE detected in the sera
|
|
• 3-fold reduction in sera levels
|
|
• no splenic NK killing activity is observed against a NK-sensitive target cell line
|
|
• Peyer's patchers are not observed
|
hematopoietic system
|
• splenic T cells do not proliferate in response to Con A
|
|
• 22-fold reduction in cellularity
(J:22521)
• 10-fold less cellularity of thymus from fetal day E15.5 through birth
(J:111821)
• 20-fold less cellularity by six days of age
(J:111821)
|
|
• thymocytes do not activate in response to Con A
|
|
• in vitro class switch using LPS and IL-4 fail to generate more IgG1-positive cells
|
|
• complete absence of gamma-delta T cells in adult thymus
• few gamma-delta T cells found in fetal thymus
• TCR-gamma rearrangements are severely reduced
|
|
• 12-fold reduction in the spleen in 3-4 week old mice
• further loss occurs with age
|
|
• plasma cells in the gut are exceptionally rare
|
|
• 20-fold decrease in bone marrow though some cells are still present
|
|
• NK cells are not detected in the spleen
|
|
• 3 fold reduction of mature alphabeta T cells in the spleen
• reduction of T cells in lamina propia of gut is observed
|
|
• no gamma-delta T cells are found in peripheral lymphnoid organs or skin
|
|
• 12-fold reduction in number of T cells found in gut epithelium
|
|
• 8-fold reduction in cellularity
|
|
• no germinal centers are observed
|
|
• clusters of white pulp are diminished in number and size
|
|
• no IgE detected in the sera
|
|
• 3-fold reduction in sera levels
|
|
• no splenic NK killing activity is observed against a NK-sensitive target cell line
|
endocrine/exocrine glands
|
• 22-fold reduction in cellularity
(J:22521)
• 10-fold less cellularity of thymus from fetal day E15.5 through birth
(J:111821)
• 20-fold less cellularity by six days of age
(J:111821)
|
|
• thymocytes do not activate in response to Con A
|
cellular
|
• splenic T cells do not proliferate in response to Con A
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
NOT | combined T cell and B cell immunodeficiency | DOID:628 | J:22521 | |
NOT | X-linked severe combined immunodeficiency | DOID:0060013 |
OMIM:300400 |
J:22521 |