mortality/aging
• most die 1-5 days after birth
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cardiovascular system
• prior to lymphatic vessel development
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• at E13.5
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hemorrhage
(
J:29797
)
• embryos at E14 show severe systemic hemorrhage which persists until E18 and then gradually recedes so that when mutants are born, they show a much reduced hemorrhage pattern than embryos
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homeostasis/metabolism
• embryos at E14 have edema that persists until E18 and then gradually recedes
|
immune system
• exhibit impaired differentiation of B-lineage cells by disrupting the signaling from the pre-BCR complex and thus preventing the clonal expansion and further maturation of pre-B cells
• mutant hematopoietic stem cells fail to produce mature B220+IgM- B cells in the spleen and peritoneal CD5+ B cells when transferred to immunodeficient rag-2 mutant recipients
• clonal expansion and maturation of pre-B cells is impaired
|
• number of cells in the small late pre-B population is drastically reduced in immunodeficient rag-2 mutant recipients that are transferred with mutant hematopoietic stem cells, indicating that B-cell development is arrested at the late pro-B stage
|
• when mutant hematopoietic stem cells are transferred into immunodeficient rag-2 mutant recipients, most B-lineage cells are arrested at the CD43+ pro-B stage and only a small proportion of the pre-B cells become B220hiCD43lo; in the most severe cases, the CD43lo population is completely absent and no B220+IgM+ cells are detected
|
• mice exhibit blood-lymphatic mixing unlike in wild-type mice
|
• overgrowing lymphatic endothelium appears to wrap around blood vessels unlike in wild-type mice
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• at E13.5, skin blood and lymphatic vessels are dilated compared to in wild-type mice
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hematopoietic system
• exhibit impaired differentiation of B-lineage cells by disrupting the signaling from the pre-BCR complex and thus preventing the clonal expansion and further maturation of pre-B cells
• mutant hematopoietic stem cells fail to produce mature B220+IgM- B cells in the spleen and peritoneal CD5+ B cells when transferred to immunodeficient rag-2 mutant recipients
• clonal expansion and maturation of pre-B cells is impaired
|
• number of cells in the small late pre-B population is drastically reduced in immunodeficient rag-2 mutant recipients that are transferred with mutant hematopoietic stem cells, indicating that B-cell development is arrested at the late pro-B stage
|
• when mutant hematopoietic stem cells are transferred into immunodeficient rag-2 mutant recipients, most B-lineage cells are arrested at the CD43+ pro-B stage and only a small proportion of the pre-B cells become B220hiCD43lo; in the most severe cases, the CD43lo population is completely absent and no B220+IgM+ cells are detected
|
limbs/digits/tail
• born with red swollen foot pads
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nervous system