hematopoietic system
• discoid platelets have enlarged diameter compared to wild-type
• platelets display 2-fold increase in number of microtubules
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• platelet counts are reduced by 73%
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homeostasis/metabolism
• platelet adhesion from blood at high shear forces (3000 s-1) is nearly abolished (83% decrease) relative to wild-type blood; platelet adhesion and aggregation is 50-60% of wild-type at a shear rate of 1500 s-1
• in vivo, application of FeCl3 produces stable occlusion in 62.5% of arteries after 30 minutes compared to 75% in wild-type; thrombi are less extensive than wild-type with little sign of degranulation
• laser beam-induced superficial lesions of mesenteric arterioles results in thrombi with surface area decreased by 45% compared to wild-type while initial thrombus growth after severe lesion is delayed but reaches comparable size at later times
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• 52% of mice bled for more than 20 minutes compared to 82% of wild-type having bleeding times of <12 minutes
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Bernard-Soulier syndrome | DOID:2217 |
OMIM:231200 |
J:135065 |