Visualizing the von Willebrand factor/glycoprotein Ib-IX axis with a platelet-type von Willebrand disease mutation.

摘要: Platelet-type von Willebrand disease (PT-VWD) is a bleeding disorder of the platelet glycoprotein Ib-IX/von factor (VWF) axis caused by mutations in Ib-IX receptor that lead to an increased affinity with VWF. In this report, platelets from mouse expressing mutation associated PT-VWD have been visualized using state-of-the art image collection and processing. Confocal analysis revealed VWF bound surface single bridging micro-aggregates platelets. Surface-bound appears as large, linear structure on 50% vivo thrombus formation after chemical injury carotid artery severe impairment occlusion consequence mutation. vitro stimulation adenosine diphosphate or thrombin demonstrates significant block their ability bind fibrinogen. The fibrinogen binding are more than might be expected observed polymers over small portion plasma membrane. Visualization receptor/ligand interaction characterization antithrombotic phenotype provide new understanding molecular basis phenotype.