mortality/aging
• homozygotes typically die withing 9 days of birth
|
growth/size/body
• pups display severe runting
|
• after 3 days, pups stop gaining weight and at 6 days weight is ~ one third of control littermates
|
cellular
• pups show elevated NF-kappaB transcription in all organs examined
|
• although there are fewer T cells, there is a higher proportion of mature CD8+ cells
|
• when exposed to B cell-specific stimuli, splenic B cells show 2- to 4-fold greater proliferation ex vivo compared to wild-type
|
• when exposed to T cell-specific stimuli, splenic T cells show a 50% reduction in proliferation compared to wild-type
|
immune system
• when exposed to B cell-specific stimuli, splenic B cells show 2- to 4-fold greater proliferation ex vivo compared to wild-type
|
• when exposed to T cell-specific stimuli, splenic T cells show a 50% reduction in proliferation compared to wild-type
|
• mice have thymic atrophy
|
• total cell number of thymocytes is dramatically decrease
|
• there are fewer B cells in the spleen, but those are more differentiated than their counterparts
|
• numbers are reduced in the thymus
|
• numbers of single positive (CD4+ or CD8+) T cells are increased in the thymus
|
small spleen
(
J:110817
)
• spleens appear smaller than in wild-type
|
• recipients of mutant fetal spleen cells show lack of germinal center formation
|
• 7 weeks after fetal liver cell transfer, recipients were challenged with OVA and OVA-specific antibody production is reduced in recipients of Nfkbia-null cells
|
• recipients of Nfkbia-null fetal liver cells by adoptive transfer show a 10-fold increase in serum levels of IgA
|
• recipients of Nfkbia-null fetal liver cells by adoptive transfer show a 10-fold increase in serum levels of IgE
|
• recipients of Nfkbia-null fetal liver cells by adoptive transfer show a 10-fold increase in serum levels of IgG2a and a 3-fold increase in IgG1 levels
|
hematopoietic system
• when exposed to B cell-specific stimuli, splenic B cells show 2- to 4-fold greater proliferation ex vivo compared to wild-type
|
• when exposed to T cell-specific stimuli, splenic T cells show a 50% reduction in proliferation compared to wild-type
|
• mice have thymic atrophy
|
• total cell number of thymocytes is dramatically decrease
|
• there are fewer B cells in the spleen, but those are more differentiated than their counterparts
|
• numbers are reduced in the thymus
|
• numbers of single positive (CD4+ or CD8+) T cells are increased in the thymus
|
small spleen
(
J:110817
)
• spleens appear smaller than in wild-type
|
• recipients of mutant fetal spleen cells show lack of germinal center formation
|
• recipients of Nfkbia-null fetal liver cells by adoptive transfer show a 10-fold increase in serum levels of IgA
|
• recipients of Nfkbia-null fetal liver cells by adoptive transfer show a 10-fold increase in serum levels of IgE
|
• recipients of Nfkbia-null fetal liver cells by adoptive transfer show a 10-fold increase in serum levels of IgG2a and a 3-fold increase in IgG1 levels
|
integument
reddish skin
(
J:110817
)
• skin of pups is red
|
scaly skin
(
J:110817
)
• pups have flaky skin
|
endocrine/exocrine glands
• mice have thymic atrophy
|
• total cell number of thymocytes is dramatically decrease
|