Analysis Tools
growth/size/body
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• E15.5 fetuses are sometimes small in size
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hematopoietic system
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• megakaryopoiesis is severerly affected
• analysis of in vitro colony formation from mononuclear ells indicates that hematopoietic progenitor formation is normal, however AChE+ cells of CFU-Megs are larger indicating dysregulation of megakaryopoiesis
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• megakaryocytes are larger in mutant fetuses than in wild-type
• megakaryocytes in the spleen are larger than in wild-type spleen
• megakaryocytes contain a large volume of cytoplasm with a paucity of cytoplasmic organelles
• demarcation membrane is underdeveloped in megakaryocytes
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• impaired megakaryocyte differentiation
• mutants exhibit dysregulated proliferation and impaired cytoplasmic maturation of megakaryocytes in adulthood
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• number of megakaryocytes is increased in E17.5 mutant livers and in hematopoietic tissues of adults
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• peripheral erythrocytes are morphologically abnormal
• in fetuses, circulation of nucleated erythroid cells is often observed whereas in wild-type mice, erythrocytes are uniformly enucleated
• enucleated erythroid cells are rarely seen in the fetal liver
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• mutants require longer time (11 days versus 7 days for wild-type mice) to recover from PHZ-induced anemia, indicating attenuated stress erythropoiesis
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anisocytosis
(
J:89054
)
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• gross morphologic abnormalities of platelets are seen
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• proplatelet formation is impaired in the bone marrow cells of mutants
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• mutants exhibit severe thrombocytopenia without anemia
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• mutants exhibit an increased number of large mononuclear cells in the bone marrow
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immune system
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• proplatelet formation is impaired in the bone marrow cells of mutants
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• mutants exhibit an increased number of large mononuclear cells in the bone marrow
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integument
cellular
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• impaired megakaryocyte differentiation
• mutants exhibit dysregulated proliferation and impaired cytoplasmic maturation of megakaryocytes in adulthood
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| thrombocytopenia | DOID:1588 |
OMIM:188000 OMIM:273900 OMIM:300367 OMIM:313900 OMIM:612004 |
J:89054 | |
