GP1b-IX-V 激活信号传导
中文名称
通路描述
血小板 GPIb 复合物 (GP1b-IX-V) 与 GPVI 主要负责调节血小板与受损血管和血小板的初始粘附及激活。GPIb 的重要性通过 Bernard-Soulier 综合征患者中的出血问题得到证明，其中该受体缺失或功能缺陷。GP1b-IX-V 与 vWF 结合于静止血小板，特别是在高剪切应力条件下。这种短暂的相互作用是血管修复过程的第一个阶段。在动脉粥样硬化斑块破裂后暴露的纤维基质或闭塞动脉上激活 GP1b-IX-V 是血栓形成的主要贡献因素，导致心脏病或中风。GpIb 还结合凝血酶 (Yamamoto 等，1986)，结合位点不同于 vWF 结合位点，作为凝血酶的结合位点，然后激活蛋白酶激活受体，导致血小板激活增强 (Dormann 等，2000)。
英文描述
GP1b-IX-V activation signalling The platelet GPIb complex (GP1b-IX-V) together with GPVI are primarily responsible for regulating the initial adhesion of platelets to the damaged blood vessel and platelet activation. The importance of GPIb is demonstrated by the bleeding problems in patients with Bernard-Soulier syndrome where this receptor is either absent or defective. GP1b-IX-V binds von Willebrand Factor (vWF) to resting platelets, particularly under conditions of high shear stress. This transient interaction is the first stage of the vascular repair process. Activation of GP1b-IX-V on exposure of the fibrous matrix following atherosclerotic plaque rupture, or in occluded arteries, is a major contributory factor leading to thrombus formation leading to heart attack or stroke. GpIb also binds thrombin (Yamamoto et al. 1986), at a site distinct from the site of vWF binding, acting as a docking site for thrombin which then activates Proteinase Activated Receptors leading to enhanced platelet activation (Dormann et al. 2000).
所含基因
12 个基因