mortality/aging
• about 30% die between E18.5 and birth
|
nervous system
• at E9.5 increased apoptosis is seen in the neuronal epithelium in the hindbrain; however, apoptosis is similar to wild-type in the forebrain, midbrain, and hindbrain at E11.5 - E16.5
|
• seen in about 30% of embryos only in the hindbrain resulting in exencephaly
|
exencephaly
(
J:67297
)
• seen in about 30% before birth
|
immune system
• total thymocyte numbers are decreased by about 25%; however B cell numbers appear similar to wild-type
|
• overall double negative thymocyte numbers are increased
• the number of CD25-CD44- double negative cells past the beta checkpoint is increased 4- to 5-fold and about 40% of these cells are in the early stages of apoptosis compared to wild-type mice where fewer than 5% of thymocytes appear apoptotic
|
• seen in the spleen and lymph nodes
|
• in purified B cells in response to B cell receptor activation resulting from a failure of cells to enter S phase
(J:67297)
• however, LPS induced proliferation is similar to wild-type
(J:67297)
• splenic B cells do not proliferate in response to anti-IgM stimulation
(J:109545)
|
• decreased basal levels of all Ig isotypes
|
• undetectable at 12 days after injection of vesicular stomatitis virus
|
• at 4 days after injection of vesicular stomatitis virus
|
• following injection of lymphocytic choriomeningitis virus the CTL memory response is absent or reduced
|
• purified T cells fail to secrete IL-2 in response to T cell receptor activation
|
• in purified T cells in response to T cell receptor activation resulting from a failure of cells to enter S phase
(J:67297)
• addition of exogenous IL-2 induces only a marginal increase in proliferation
(J:67297)
• purified T lymphocytes fail to expand or produce Il2when stimulated with antiCD3 +/- antiCD28 costimulation; they do not respond to stimulation with PMA
(J:109545)
|
• impaired zymosan-induced production of Tnfa in bone marrow dendritic cells
• impaired fungal cell-induced production of Tnfa, Il2, and Il10 in bone marrow dendritic cells
|
• following injection of lymphocytic choriomeningitis virus the initial swelling reaction of the foot pad is reduced and CTL memory response is absent or reduced
• following injection of vesicular stomatitis virus the antigen specific IgM response is reduced (at 4 days) and no IgG response is detected (at 12 days)
|
cellular
N |
• induced-apoptosis in ES cells, MEFs, and thymocytes is similar to wild-type
|
• at E9.5 increased apoptosis is seen in the neuronal epithelium in the hindbrain; however, apoptosis is similar to wild-type in the forebrain, midbrain, and hindbrain at E11.5 - E16.5
|
• in purified B cells in response to B cell receptor activation resulting from a failure of cells to enter S phase
(J:67297)
• however, LPS induced proliferation is similar to wild-type
(J:67297)
• splenic B cells do not proliferate in response to anti-IgM stimulation
(J:109545)
|
• in purified T cells in response to T cell receptor activation resulting from a failure of cells to enter S phase
(J:67297)
• addition of exogenous IL-2 induces only a marginal increase in proliferation
(J:67297)
• purified T lymphocytes fail to expand or produce Il2when stimulated with antiCD3 +/- antiCD28 costimulation; they do not respond to stimulation with PMA
(J:109545)
|
hematopoietic system
• in purified B cells in response to B cell receptor activation resulting from a failure of cells to enter S phase
(J:67297)
• however, LPS induced proliferation is similar to wild-type
(J:67297)
• splenic B cells do not proliferate in response to anti-IgM stimulation
(J:109545)
|
• total thymocyte numbers are decreased by about 25%; however B cell numbers appear similar to wild-type
|
• overall double negative thymocyte numbers are increased
• the number of CD25-CD44- double negative cells past the beta checkpoint is increased 4- to 5-fold and about 40% of these cells are in the early stages of apoptosis compared to wild-type mice where fewer than 5% of thymocytes appear apoptotic
|
• seen in the spleen and lymph nodes
|
• decreased basal levels of all Ig isotypes
|
• undetectable at 12 days after injection of vesicular stomatitis virus
|
• at 4 days after injection of vesicular stomatitis virus
|
• following injection of lymphocytic choriomeningitis virus the CTL memory response is absent or reduced
|
• purified T cells fail to secrete IL-2 in response to T cell receptor activation
|
• in purified T cells in response to T cell receptor activation resulting from a failure of cells to enter S phase
(J:67297)
• addition of exogenous IL-2 induces only a marginal increase in proliferation
(J:67297)
• purified T lymphocytes fail to expand or produce Il2when stimulated with antiCD3 +/- antiCD28 costimulation; they do not respond to stimulation with PMA
(J:109545)
|
embryo
• at E9.5 the neural folds at the hindbrain fail to elevate on either side of the midline and do not bend toward each other
|
• seen in about 30% of embryos only in the hindbrain resulting in exencephaly
|
endocrine/exocrine glands
• total thymocyte numbers are decreased by about 25%; however B cell numbers appear similar to wild-type
|