VWF 靶点信息

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。

VWF, von Willebrand factor 分子靶点种属信息

人 Human小鼠 Mouse大鼠 Rat

人 Human VWF 分子靶点信息

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).

小鼠 Mouse Vwf 分子靶点信息

小鼠 Mouse Vwf 靶点分子功能（预测）

Predicted to enable several functions, including chaperone binding activity; identical protein binding activity; and integrin binding activity. Acts upstream of or within several processes, including liver development; placenta development; and platelet activation. Located in external side of plasma membrane. Is expressed in several structures, including cardiovascular system; extraembryonic component; genitourinary system; leptomeninges; and vitelline blood vessel. Used to study von Willebrand's disease and von Willebrand's disease 2. Human ortholog(s) of this gene implicated in several diseases, including Behcet's disease; Bernard-Soulier syndrome; end stage renal disease; essential thrombocythemia; and von Willebrand's disease (multiple). Orthologous to human VWF (von Willebrand factor).

大鼠 Rat Vwf 分子靶点信息

大鼠 Rat Vwf 靶点分子功能（预测）

Predicted to enable several functions, including chaperone binding activity; identical protein binding activity; and integrin binding activity. Involved in several processes, including liver regeneration; platelet activation; and response to L-ascorbic acid. Located in collagen-containing extracellular matrix; endoplasmic reticulum; and extracellular space. Used to study transient cerebral ischemia. Biomarker of hypertension; mesangial proliferative glomerulonephritis; myocardial infarction; pulmonary fibrosis; and type 1 diabetes mellitus. Human ortholog(s) of this gene implicated in several diseases, including Behcet's disease; Bernard-Soulier syndrome; end stage renal disease; essential thrombocythemia; and von Willebrand's disease (multiple). Orthologous to human VWF (von Willebrand factor).

VWF 分子产品列表

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