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Phenotypes Associated with This Genotype
Genotype
MGI:3714069
Allelic
Composition
Rag2tm1Avla/Rag2tm1Avla
Genetic
Background
involves: 129/Sv * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rag2tm1Avla mutation (0 available); any Rag2 mutation (64 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• develops in 4% of mice, the most severe phenotype group (J:122108)
• develops in 4% of mice, the most severe phenotype group (J:122108)
• severe lymphocyte infiltration of the lungs and liver occurs (J:122108)
• severe lymphocyte infiltration of the lungs and liver occurs (J:122108)
• mice challenged with ovalbumin fail to produce ovalbumin-specific antibodies despite low, but detectible, Ig levels (J:122108)
• mice challenged with ovalbumin fail to produce ovalbumin-specific antibodies despite low, but detectible, Ig levels (J:122108)
• decreased proliferative response in response to anti-CD3 antibody stimulation (J:122108)
• however, response to phorbol 12-myristate/ionomycin stimulation was normal (J:122108)
• decreased proliferative response in response to anti-CD3 antibody stimulation (J:122108)
• however, response to phorbol 12-myristate/ionomycin stimulation was normal (J:122108)
• development of B lymphocytes is impaired with no immature or mature recirculating B cell detected (J:122108)
• development of B lymphocytes is impaired with no immature or mature recirculating B cell detected (J:122108)
• thymocytes do not developed past the DN3 stage (J:122108)
• thymocytes do not developed past the DN3 stage (J:122108)
• at 6 to 8 weeks, decrease in lymphoid cells in the thymus, lymph nodes and spleen (J:122108)
• at 6 to 8 weeks, decrease in lymphoid cells in the thymus, lymph nodes and spleen (J:122108)
• in thymus, spleen, mesenteric, inguinal and cervical lymph nodes (J:122108)
• in thymus, spleen, mesenteric, inguinal and cervical lymph nodes (J:122108)
• NKT (natural killer T) cells are decreased in the thymus, spleen, mesenteric, inguinal, and cervical lymph nodes (J:122108)
• NKT (natural killer T) cells are decreased in the thymus, spleen, mesenteric, inguinal, and cervical lymph nodes (J:122108)
• the CD4+CD8+ double-positive compartment is depleted while the double negative compartment is enriched (J:122108)
• the CD4+CD8+ double-positive compartment is depleted while the double negative compartment is enriched (J:122108)
• at 6 to 8 weeks, thymi are deficient in Hassall corpuscle-like clusters (J:122108)
• at 6 to 8 weeks, thymi are deficient in Hassall corpuscle-like clusters (J:122108)
• at 6 to 8 weeks (J:122108)
• at 6 to 8 weeks (J:122108)
• at 6 to 8 weeks, severe depletion of white matter and B cells in the spleen (J:122108)
• at 6 to 8 weeks, severe depletion of white matter and B cells in the spleen (J:122108)
• at 6 to 8 weeks, abnormal architecture with a severe depletion in B cells and a lack of B follicles (J:122108)
• at 6 to 8 weeks, abnormal architecture with a severe depletion in B cells and a lack of B follicles (J:122108)
• mesenteric, inguinal, and cervical lymph nodes are small (J:122108)
• mesenteric, inguinal, and cervical lymph nodes are small (J:122108)
• 4% of mice develop skin erythroderma (J:122108)
• mice show increased infiltration of T lymphocytes and eosinophils in the skin (J:122108)
• 4% of mice develop skin erythroderma (J:122108)
• mice show increased infiltration of T lymphocytes and eosinophils in the skin (J:122108)

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 (J:122108)
• 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 (J:122108)
• develops in 4% of mice, the most severe phenotype group (J:122108)
• develops in 4% of mice, the most severe phenotype group (J:122108)

growth/size/body
• develops in 4% of mice (J:122108)
• develops in 4% of mice (J:122108)

hematopoietic system
• the CD4+CD8+ double-positive compartment is depleted while the double negative compartment is enriched (J:122108)
• the CD4+CD8+ double-positive compartment is depleted while the double negative compartment is enriched (J:122108)
• at 6 to 8 weeks, thymi are deficient in Hassall corpuscle-like clusters (J:122108)
• at 6 to 8 weeks, thymi are deficient in Hassall corpuscle-like clusters (J:122108)
• development of B lymphocytes is impaired with no immature or mature recirculating B cell detected (J:122108)
• development of B lymphocytes is impaired with no immature or mature recirculating B cell detected (J:122108)
• thymocytes do not developed past the DN3 stage (J:122108)
• thymocytes do not developed past the DN3 stage (J:122108)
• at 6 to 8 weeks, decrease in lymphoid cells in the thymus, lymph nodes and spleen (J:122108)
• at 6 to 8 weeks, decrease in lymphoid cells in the thymus, lymph nodes and spleen (J:122108)
• in thymus, spleen, mesenteric, inguinal and cervical lymph nodes (J:122108)
• in thymus, spleen, mesenteric, inguinal and cervical lymph nodes (J:122108)
• NKT (natural killer T) cells are decreased in the thymus, spleen, mesenteric, inguinal, and cervical lymph nodes (J:122108)
• NKT (natural killer T) cells are decreased in the thymus, spleen, mesenteric, inguinal, and cervical lymph nodes (J:122108)
• at 6 to 8 weeks (J:122108)
• at 6 to 8 weeks (J:122108)
• at 6 to 8 weeks, severe depletion of white matter and B cells in the spleen (J:122108)
• at 6 to 8 weeks, severe depletion of white matter and B cells in the spleen (J:122108)
• severe lymphocyte infiltration of the lungs and liver occurs (J:122108)
• severe lymphocyte infiltration of the lungs and liver occurs (J:122108)
• mice challenged with ovalbumin fail to produce ovalbumin-specific antibodies despite low, but detectible, Ig levels (J:122108)
• mice challenged with ovalbumin fail to produce ovalbumin-specific antibodies despite low, but detectible, Ig levels (J:122108)
• decreased proliferative response in response to anti-CD3 antibody stimulation (J:122108)
• however, response to phorbol 12-myristate/ionomycin stimulation was normal (J:122108)
• decreased proliferative response in response to anti-CD3 antibody stimulation (J:122108)
• however, response to phorbol 12-myristate/ionomycin stimulation was normal (J:122108)

integument
• 4% of mice develop skin erythroderma (J:122108)
• mice show increased infiltration of T lymphocytes and eosinophils in the skin (J:122108)
• 4% of mice develop skin erythroderma (J:122108)
• mice show increased infiltration of T lymphocytes and eosinophils in the skin (J:122108)
• 60% of mice develop substantial dorsal and facial hair loss (J:122108)
• hair loss is severe in 4% of mice (J:122108)
• 60% of mice develop substantial dorsal and facial hair loss (J:122108)
• hair loss is severe in 4% of mice (J:122108)

endocrine/exocrine glands
• the CD4+CD8+ double-positive compartment is depleted while the double negative compartment is enriched (J:122108)
• the CD4+CD8+ double-positive compartment is depleted while the double negative compartment is enriched (J:122108)
• at 6 to 8 weeks, thymi are deficient in Hassall corpuscle-like clusters (J:122108)
• at 6 to 8 weeks, thymi are deficient in Hassall corpuscle-like clusters (J:122108)

Mouse Models of Human Disease
OMIM ID Ref(s)
Omenn Syndrome 603554 J:122108


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
01/26/2016
MGI 6.02
The Jackson Laboratory