Laboratory for Thrombosis and Haemostasis, Department of Clinical Chemistry & Haematology, University Medical Center Utrecht, Utrecht, The Netherlands.
Haemophilia. 2010 May;16(102):6-15. doi: 10.1111/j.1365-2516.2008.01864.x. Epub 2008 Sep 1.
Factor VIII (FVIII) is a plasma protein critical to the haemostatic system. This notion is illustrated by the severe bleeding disorder that is associated with its functional absence, known as haemophilia A. In addition, several epidemiological studies have revealed an association between the presence of elevated levels of FVIII and thrombotic complications. In view of its relation to thrombotic and haemorrhagic disorders, it is not surprising that FVIII has gained wide attention from the research community in the previous decades. This research has led to a better understanding of not only the structural, functional and physiological aspects of this intriguing protein, but also of the pathogenesis of haemostatic defects associated with FVIII. In the present review, focus will be on the interaction between FVIII and surface receptors that are able to capture FVIII. These interactions are of importance for FVIII, as they may affect both function and survival of FVIII.
VIII 因子(FVIII)是一种对止血系统至关重要的血浆蛋白。这种观点可以通过其功能缺失所导致的严重出血性疾病得到说明,这种疾病被称为血友病 A。此外,几项流行病学研究揭示了 FVIII 水平升高与血栓并发症之间存在关联。鉴于其与血栓形成和出血性疾病的关系,FVIII 在过去几十年中引起了研究界的广泛关注也就不足为奇了。这项研究不仅使人们对这种有趣的蛋白质的结构、功能和生理方面有了更好的理解,也使人们对与 FVIII 相关的止血缺陷的发病机制有了更好的理解。在本次综述中,重点将放在 FVIII 与能够捕获 FVIII 的表面受体之间的相互作用上。这些相互作用对 FVIII 很重要,因为它们可能会影响 FVIII 的功能和存活。