Analysis Tools
hematopoietic system
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• approximately 30% of megakaryocytes are smaller than 50 um2 compared to about 4% in wild-type
• many megakaryocytes exhibit an irregular morphology
• however, megakaryocytes show normal distribution and morphology of F-actin and tubulin
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• mice show heterogeneity in the size and morphology of platelets, with frequent vacuoles, indicating increased fragility of platelets
• nonstimulated platelets exhibit a reduction in F-actin levels
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• proplatelet formation is reduced by approximately 40% in fetal liver-derived megakaryocytes
• while initial morphological changes associated with proplatelet formation occur, megakaryocytes exhibit few or no branches
• where proplatelets do form, their tips are increased in size relative to wild-type
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• mice exhibit a more severe form of macrothrombocytopenia than in heterozygotes, with an approximate 50% reduction in platelet counts
• transplantation of unfractionated bone marrow from homozygotes into lethally irradiated adult recipients results in macrothrombocytopenia
• however, other peripheral blood cell counts are unchanged
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• modest, but significant, increase in lymphocyte counts
• however, bone marrow shows normal numbers of immunophenotypic hematopoietic progenitor cells, including megakaryocyte progenitors
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• thrombin-activated platelets on fibrinogen-coated coverslips fully spread but the spreading process is delayed
• thrombus formation on collagen under flow ex vivo is reduced in blood of mutants
• however, platelet turnover is unaffected and platelets are responsive to a range of agonist, indicating that the processes of integrin inside-out activation and degranulation are function
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homeostasis/metabolism
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• thrombin-activated platelets on fibrinogen-coated coverslips fully spread but the spreading process is delayed
• thrombus formation on collagen under flow ex vivo is reduced in blood of mutants
• however, platelet turnover is unaffected and platelets are responsive to a range of agonist, indicating that the processes of integrin inside-out activation and degranulation are function
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• tail bleeding times are mildly extended in homozygotes and in lethally irradiated mice with transplanted homozygous bone marrow
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immune system
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• proplatelet formation is reduced by approximately 40% in fetal liver-derived megakaryocytes
• while initial morphological changes associated with proplatelet formation occur, megakaryocytes exhibit few or no branches
• where proplatelets do form, their tips are increased in size relative to wild-type
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• modest, but significant, increase in lymphocyte counts
• however, bone marrow shows normal numbers of immunophenotypic hematopoietic progenitor cells, including megakaryocyte progenitors
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