Human von Willebrand Factor Sdandard

VWF 分子靶点信息概述

• 分子名：VWF, von Willebrand factor
• 基因家族：Receptor ligands
• 曾用名：F8VWF
• 全称：Factor VIII related antigen

VWF 分子靶点综述

血管性血友病因子(vWF)是一种存在于血浆中的多聚体糖蛋白，在内皮(Weibel-Palade小体)、巨核细胞(血小板α-颗粒)和内皮下结缔组织中可生成超大vWF。vWF多聚体分子量非常大(大于20,000kDa)，由超过80个分子量为250kDa的亚基组成。vWF的主要功能是结合其它蛋白特别是VIII因子，在血小板粘附到伤口部位中起重要作用，另外，它在凝血过程中也起着重要作用。因此，vWF缺失或者功能障碍(血管性血友病)将导致出血倾向，在窄血管中有高血流量的组织中最明显。监测血清中vWF水平可为一些病理情况如血栓性血小板减少性紫癜、海德综合征、可能是溶血性尿毒症综合征提供更详细的见解。

人 Human VWF 分子靶点信息

• 分子名：VWF, von Willebrand factor
• 别称：
  • coagulation factor VIII VWF
  • F8VWF
  • VWD
• 基因序列：NCBI_Gene: 7450
• 蛋白序列：UniProtKB: P04275

人 Human VWF靶点分子功能（预测）

Enables several functions, including chaperone binding activity; identical protein binding activity; and integrin binding activity. Involved in cell-substrate adhesion; platelet activation; and positive regulation of intracellular signal transduction. Located in several cellular components, including Weibel-Palade body; collagen-containing extracellular matrix; and endoplasmic reticulum. Implicated in several diseases, including Behcet's disease; Bernard-Soulier syndrome; end stage renal disease; essential thrombocythemia; and von Willebrand's disease (multiple). Biomarker of several diseases, including end stage renal disease; familial combined hyperlipidemia; obesity; osteonecrosis; and von Willebrand's disease (multiple).