Analysis Tools
immune system
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• thymus showed absence of the lymphocytic cortex, and predominance of connective tissue with scattered lymphoid cells
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• thymus weighted approximately 20% of wildtype and was poorly developed at 2 and 4-5 weeks of age
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• arrest of B cell development at an early progenitor stage
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• more than 90% of thymocytes were arrested at the CD4-CD8- stage, compared to 5-10% arrested in wildtype, there were at least 20 times more CD4-CD8-CD25+ cells and showed near absence of CD8+CD3+ and CD4+CD3+ mature single-positive cells in 2 week old mutants
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• lymphoid depletion
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• thymoctye counts were 0.7-4% of that observed in wildtype
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small spleen
(
J:96535
)
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• spleen was smaller and poorly developed at 2 and 4-5 weeks of age
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• spleens showed reduction in fully developed lymphoid follicles, in which majority of lymphoid cells showed larger nuclei with less dense chromatin and prominent nucleoli
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• impaired coding joint rearrangement (10 fold decrease in DH-JH2 and 100 fold decrease in VH7183-JH2 rearrangement and 10-100 fold reduction in TCRbeta and Ddelta2-Jdelta1 coding joint formation) while retaining intact signal ends and close to normal signal joint formation
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• B cell proliferation to LPS was 13.5% of that in wildtype
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• T cell proliferation to Con A and anti-CD3 was reduced to 16.5 and 18.7% of that seen in wildtype
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• lymph nodes were severely depleted of mature lymphocytes, with complete absence of lymphoid follicles
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• lymph nodes were smaller and poorly developed at 2 and 4-5 weeks of age
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hematopoietic system
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• thymus showed absence of the lymphocytic cortex, and predominance of connective tissue with scattered lymphoid cells
|
|
• thymus weighted approximately 20% of wildtype and was poorly developed at 2 and 4-5 weeks of age
|
|
• arrest of B cell development at an early progenitor stage
|
|
• more than 90% of thymocytes were arrested at the CD4-CD8- stage, compared to 5-10% arrested in wildtype, there were at least 20 times more CD4-CD8-CD25+ cells and showed near absence of CD8+CD3+ and CD4+CD3+ mature single-positive cells in 2 week old mutants
|
|
• lymphoid depletion
|
|
• thymoctye counts were 0.7-4% of that observed in wildtype
|
small spleen
(
J:96535
)
|
• spleen was smaller and poorly developed at 2 and 4-5 weeks of age
|
|
• spleens showed reduction in fully developed lymphoid follicles, in which majority of lymphoid cells showed larger nuclei with less dense chromatin and prominent nucleoli
|
|
• impaired coding joint rearrangement (10 fold decrease in DH-JH2 and 100 fold decrease in VH7183-JH2 rearrangement and 10-100 fold reduction in TCRbeta and Ddelta2-Jdelta1 coding joint formation) while retaining intact signal ends and close to normal signal joint formation
|
|
• B cell proliferation to LPS was 13.5% of that in wildtype
|
|
• T cell proliferation to Con A and anti-CD3 was reduced to 16.5 and 18.7% of that seen in wildtype
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cellular
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• coding joint formation was undetectable in MEFs in an extrachromosomal V(D)J recombination assay
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• MEFs exhibit elevated sensitivity to ionizing radiation
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• B cell proliferation to LPS was 13.5% of that in wildtype
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• T cell proliferation to Con A and anti-CD3 was reduced to 16.5 and 18.7% of that seen in wildtype
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endocrine/exocrine glands
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• thymus showed absence of the lymphocytic cortex, and predominance of connective tissue with scattered lymphoid cells
|
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• thymus weighted approximately 20% of wildtype and was poorly developed at 2 and 4-5 weeks of age
|
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• thymoctye counts were 0.7-4% of that observed in wildtype
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| severe combined immunodeficiency with sensitivity to ionizing radiation | DOID:0090012 |
OMIM:602450 |
J:96535 | |
