hematopoietic system
• homozygotes display a generally normal hematological profile, including normal platelet count, plasma fibrinogen level, clotting time and fibrin crosslinking
• however, in vitro, plasma suspensions from mutant mice fail to exhibit ADP (or collagen)-induced platelet aggregation, despite an observable platelet shape change
• addition of either normal mouse plasma or purified mouse fibrinogen restores platelet aggregation, regardless of the agonist used
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homeostasis/metabolism
• homozygotes display a generally normal hematological profile, including normal platelet count, plasma fibrinogen level, clotting time and fibrin crosslinking
• however, in vitro, plasma suspensions from mutant mice fail to exhibit ADP (or collagen)-induced platelet aggregation, despite an observable platelet shape change
• addition of either normal mouse plasma or purified mouse fibrinogen restores platelet aggregation, regardless of the agonist used
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• homozygotes exhibit significantly increased bleeding times following a standard injury to the nail bed on the 5th digit of the hindleg relative to wild-type mice (>25 min vs ~5 min, respectively)
• however, homozygotes are able to control bleeding at other sites of injury that involve small blood vessels, such as a 1.3 cm full-thickness skin incision
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cardiovascular system
• homozygotes that survive to weaning show no overt or microscopic defects in any major organ system or incidences of bleeding, illness or death up to 4 months of age
• female homozygotes are fertile and do not exhibit fatal intrauterine hemorrhage during pregnancy
• however, 4 of 173 (2.3%) of neonatal homozygotes display spontaneous, fatal bleeding into the peritoneal cavity; in one case, the source of bleeding is a ruptured blood vessel within the distended stomach
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