نتایج جستجو برای: glanzmann thrombasthenia
تعداد نتایج: 568 فیلتر نتایج به سال:
From the initial description of platelets in 1882, their propensity to aggregate and to contribute to thrombosis was apparent. Indeed, excessive platelet aggregation is associated with myocardial infarction and other thrombotic diseases whereas Glanzmann thrombasthenia, in which platelet aggregation is reduced, is a bleeding syndrome. Over the last half of the 20th century, many investigators h...
The cytoadhesin family consists of platelet glycoprotein (GP) IIb-IIIa and the endothelial vitronectin receptor. The beta subunit (GP IIIa) of these complexes expresses the alloantigen Zwa (or PIA1). This alloantigen is not expressed by members of other integrin subfamilies. By using immunoprecipitation and immunoblot techniques, we found that the beta subunit of a heterodimer, expressed by cul...
Glanzmann thrombasthenia is an inherited bleeding disorder characterized by absence or dysfunction of the platelet integrin a IIb b 3 . Patient RM is a thrombasthenic variant whose platelets fail to aggregate in response to physiological agonists, despite the fact that they express abundant levels of a IIb b 3 on their surface. Binding of soluble fibrinogen or fibrinogen mimetic antibodies to R...
Integrin αIIbβ3 is indispensable for normal hemostasis, but its role for thrombopoiesis is still controversial. Recently, αIIb and β3 mutations have been identified in patients with congenital macrothrombocytopenia. We analyzed three unrelated Japanese families with congenital macrothrombocytopenia. Expression and activation state of αIIbβ3 in platelets was examined by flow cytometry and immuno...
We have studied the pathways that lead to arrest and firm adhesion of rolling PMN on activated, surface-adherent platelets. Stable arrest and adhesion strengthening of PMN on thrombin-stimulated, surface-adherent platelets in flow required distinct Ca 2 1 and Mg 2 1 -dependent regions of Mac-1 ( a M b 2), and involved interactions of Mac-1 with fibrinogen, which was bound to platelets via a IIb...
BACKGROUND The diagnostic evaluation of inherited platelet disorders (IPDs) is complicated and time-consuming, resulting in a relevant number of undiagnosed and incorrectly classified patients. In order to evaluate the spectrum of IPDs in individuals with clinical suspicion of these disorders, and to provide a diagnostic tool to centers not having access to specific platelets studies, we establ...
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