Analysis Tools
immune system
|
• severe lymphocyte infiltration of the lungs and liver occurs
|
|
• decreased proliferative response in response to anti-CD3 antibody stimulation
• however, response to phorbol 12-myristate/ionomycin stimulation was normal
|
|
• mice challenged with ovalbumin fail to produce ovalbumin-specific antibodies despite low, but detectible, Ig levels
|
|
• development of B lymphocytes is impaired with no immature or mature recirculating B cell detected
|
|
• thymocytes do not developed past the DN3 stage
|
|
• at 6 to 8 weeks, decrease in lymphoid cells in the thymus, lymph nodes and spleen
|
|
• in thymus, spleen, mesenteric, inguinal and cervical lymph nodes
|
|
• NKT (natural killer T) cells are decreased in the thymus, spleen, mesenteric, inguinal, and cervical lymph nodes
|
|
• the CD4+CD8+ double-positive compartment is depleted while the double negative compartment is enriched
|
|
• at 6 to 8 weeks, thymi are deficient in Hassall corpuscle-like clusters
|
|
• at 6 to 8 weeks
|
small spleen
(
J:122108
)
|
• at 6 to 8 weeks, severe depletion of white matter and B cells in the spleen
|
|
• at 6 to 8 weeks, abnormal architecture with a severe depletion in B cells and a lack of B follicles
|
|
• mesenteric, inguinal, and cervical lymph nodes are small
|
erythroderma
(
J:122108
)
|
• 4% of mice develop skin erythroderma
• mice show increased infiltration of T lymphocytes and eosinophils in the skin
|
digestive/alimentary system
|
• at 6 to 8 weeks, mice show increased infiltration of T lymphocytes and eosinophils in the gut especially in the more severe group of mice
|
growth/size/body
hematopoietic system
|
• the CD4+CD8+ double-positive compartment is depleted while the double negative compartment is enriched
|
|
• at 6 to 8 weeks, thymi are deficient in Hassall corpuscle-like clusters
|
|
• development of B lymphocytes is impaired with no immature or mature recirculating B cell detected
|
|
• thymocytes do not developed past the DN3 stage
|
|
• at 6 to 8 weeks, decrease in lymphoid cells in the thymus, lymph nodes and spleen
|
|
• in thymus, spleen, mesenteric, inguinal and cervical lymph nodes
|
|
• NKT (natural killer T) cells are decreased in the thymus, spleen, mesenteric, inguinal, and cervical lymph nodes
|
|
• at 6 to 8 weeks
|
small spleen
(
J:122108
)
|
• at 6 to 8 weeks, severe depletion of white matter and B cells in the spleen
|
|
• severe lymphocyte infiltration of the lungs and liver occurs
|
|
• decreased proliferative response in response to anti-CD3 antibody stimulation
• however, response to phorbol 12-myristate/ionomycin stimulation was normal
|
|
• mice challenged with ovalbumin fail to produce ovalbumin-specific antibodies despite low, but detectible, Ig levels
|
integument
erythroderma
(
J:122108
)
|
• 4% of mice develop skin erythroderma
• mice show increased infiltration of T lymphocytes and eosinophils in the skin
|
|
• 60% of mice develop substantial dorsal and facial hair loss
• hair loss is severe in 4% of mice
|
endocrine/exocrine glands
|
• the CD4+CD8+ double-positive compartment is depleted while the double negative compartment is enriched
|
|
• at 6 to 8 weeks, thymi are deficient in Hassall corpuscle-like clusters
|
cellular
|
• decreased proliferative response in response to anti-CD3 antibody stimulation
• however, response to phorbol 12-myristate/ionomycin stimulation was normal
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Omenn syndrome | DOID:0060010 |
OMIM:603554 |
J:122108 | |
