Role of von Willebrand Factor in the Vessel Wall
Role of von Willebrand Factor in the Vessel Wall
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血管性血友病因子在血管壁中的作用
DOI：
10.1055/s-2007-1003518
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发表时间：
1987
影响因子：
5.7
通讯作者：
J. Sixma
中科院分区：
文献类型：
--
作者：
P. D. de Groot;J. Sixma
血管壁的完整性及其防止损伤后严重出血的能力都由其中存在的粘附蛋白控制，其中最令人感兴趣的蛋白质之一是血管性血友病因子（vWF），这并不是因为近年来对其结构和功能的了解太多。vWF在粘附蛋白中是例外的，因为它仅在巨核细胞和内皮细胞中合成，存在于血浆、血小板以及血管壁中，并且仅具有有限的粘附谱，即，朝向血小板。在这篇综述文章中，我们将讨论血管性血友病因子的合成内皮细胞和分泌的方式，连同一些最近的数据，这种合成和分泌是如何调节。我们将注意内皮下vWF的性质和数量。考虑vWF的功能，特别是血小板对血管壁的粘附。我们将看到血管壁中的vWF本身不足以实现最佳的血小板粘附。血浆vWF也是必需的，但它首先与血管壁结合，然后改变构象，以使血小板与之相互作用。为了了解vWF是如何在内皮细胞合成后并随后向基质分泌后掺入血管壁基质的，以及当内皮下暴露于血浆vWF时，了解vWF如何与血管壁中存在的各种物质结合是很重要的。抗vWF的单克隆抗体在鉴定vWF的特异性结合和识别vWF分子上的结合区域方面非常有帮助。最近，随着特异性单克隆抗体（Mab）的出现，蛋白水解消化的使用和氨基酸测序以鉴定特定结构域，vWF的结构-功能关系研究成为可能。分子克隆技术的使用与模拟特定功能的肽的制备相结合，将很快使这些见解降低到几个氨基酸残基的水平。
Both the integrity of the vessel wall and its capacity to prevent serious bleeding on injury are governed by the adhesive proteins present in it. One of the most interesting of these proteins is von Willebrand factor (vWF), not in the least because so much has been learned about its structure and function in recent years. vWF is exceptional among the adhesive proteins in that it is synthesized in only the megakaryocytes and the endothelial cells, present in plasma, platelets, as well as in the vessel wall, and possesses only a limited spectrum of adhesiveness, that is, toward platelets. In this review article we shall discuss the synthesis of vWF by endothelial cells and the way in which vWF is secreted, together with some recent data on how this synthesis and secretion are regulated. We shall then pay attention to the nature and quantity of vWF in the subendothelium. The function of vWF is considered, particularly the adhesion of blood platelets to the vessel wall. We will see that vWF in the vessel wall is by itself insufficient for optimal platelet adhesion. Plasma vWF is also required, but this binds first to the vessel wall and then changes in conformation in order to enable the platelet to interact with it. For the understanding of how vWF is incorporated into the vessel wall matrix both after synthesis by endothelial cells and followed by secretion toward the matrix, and when the subendothelium is exposed to plasma vWF, it is important to know how vWF binds to various substances that are present in the vessel wall. Monoclonal antibodies to vWF have been very helpful in identifying specific binding of vWF and in recognizing binding regions on the vWF molecule. Structure-function relationship studies on vWF have recently become possible with the advent of specific monoclonal antibodies (Mabs), the use of proteolytic digestion, and amino acid sequencing to identify specific domains. The use of molecular cloning in combination with preparation of peptides mimicking specified functions will soon bring these insights down to the level of a few amino acid residues.
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血管性血友病蛋白独立于胶原蛋白与细胞外基质结合。
DOI：
10.1073/pnas.81.2.471
发表时间：
1984
影响因子：
11.1
作者：
Wagner,DD;Urban-Pickering,M;Marder,VJ
通讯作者：
Marder,VJ
DOI：
10.1016/s0021-9258(17)39509-1
发表时间：
1985-07
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
H. Slayter;Joseph Loscalzo;P. Bockenstedt;R. I. Handin
通讯作者：
H. Slayter;Joseph Loscalzo;P. Bockenstedt;R. I. Handin
DOI：
10.1073/pnas.82.23.8057
发表时间：
1985-01-01
影响因子：
11.1
作者：
PLOW, EF;PIERSCHBACHER, MD;GINSBERG, MH
通讯作者：
GINSBERG, MH
内皮下因子 VIII/von Willebrand 因子介导血小板粘附。
影响因子：
20.3
作者：
Turitto,VT;Weiss,HJ;Zimmerman,TS;Sussman,II
通讯作者：
Sussman,II
冯维勒布兰德氏病血管段上血小板粘附减少：初始血小板粘附缺陷。
DOI：
--
发表时间：
1983
期刊：
The Journal of laboratory and clinical medicine
影响因子：
--
作者：
Turitto,VT;Weiss,HJ;Baumgartner,HR
通讯作者：
Baumgartner,HR