Dermatology and Venerology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany.
School of Life Sciences, University of Siegen, 57076 Siegen, Germany.
Cells. 2021 Sep 8;10(9):2351. doi: 10.3390/cells10092351.
The plasma glycoprotein von Willebrand factor (VWF) is exclusively synthesized in endothelial cells (ECs) and megakaryocytes, the precursor cells of platelets. Its primary function lies in hemostasis. However, VWF is much more than just a "fishing hook" for platelets and a transporter for coagulation factor VIII. VWF is a true multitasker when it comes to its many roles in cellular processes. In ECs, VWF coordinates the formation of Weibel-Palade bodies and guides several cargo proteins to these storage organelles, which control the release of hemostatic, inflammatory and angiogenic factors. Leukocytes employ VWF to assist their rolling on, adhesion to and passage through the endothelium. Vascular smooth muscle cell proliferation is supported by VWF, and it regulates angiogenesis. The life cycle of platelets is accompanied by VWF from their budding from megakaryocytes to adhesion, activation and aggregation until the end in apoptosis. Some tumor cells acquire the ability to produce VWF to promote metastasis and hide in a shell of VWF and platelets, and even the maturation of osteoclasts is regulated by VWF. This review summarizes the current knowledge on VWF's versatile cellular functions and the resulting pathophysiological consequences of their dysregulation.
血浆糖蛋白血管性血友病因子（VWF）仅在内皮细胞（ECs）和巨核细胞（血小板的前体细胞）中合成。其主要功能在于止血。然而，VWF 的作用远不止于作为血小板的“钓饵”和凝血因子 VIII 的载体。在细胞过程中，VWF 具有多种作用，是真正的多面手。在 ECs 中，VWF 协调 Weibel-Palade 小体的形成，并指导几种 cargo 蛋白进入这些储存细胞器，这些细胞器控制止血、炎症和血管生成因子的释放。白细胞利用 VWF 协助其滚动、黏附和穿过内皮细胞。VWF 支持血管平滑肌细胞的增殖，并调节血管生成。血小板的生命周期伴随着 VWF，从巨核细胞的萌芽到黏附、激活和聚集，直到凋亡结束。一些肿瘤细胞获得了产生 VWF 的能力，以促进转移，并隐藏在 VWF 和血小板的外壳中，甚至破骨细胞的成熟也受到 VWF 的调节。这篇综述总结了目前关于 VWF 多功能细胞功能的知识，以及它们失调导致的病理生理后果。
