抗人 von Willebrand Factor 抗体对
01AB00626
Von Willebrand factor (VWF) is a large multimeric glycoprotein essential for hemostasis, primarily facilitating platelet adhesion to sites of vascular injury. The protein's structure includes multiple binding domains that interact with platelets and collagen, critical for its hemostatic function. It exists in plasma as multimers ranging from 500 to 20,000 kDa, with larger forms being more functionally active. VWF plays a dual role by not only promoting platelet aggregation but also serving as a carrier for coagulation factor VIII, extending its half-life in circulation. Deficiencies or dysfunctions in VWF lead to von Willebrand disease (VWD), the most common inherited bleeding disorder, characterized by prolonged bleeding due to inadequate platelet function and reduced clot formation. Additionally, abnormal VWF levels can be associated with thrombotic conditions such as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), where excessive VWF leads to increased thrombosis risk.
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技术参数
- 靶点基因名
- VWF
- 基因别名
- F8VWF
- 蛋白别名
- von Willebrand factor; von Willebrand Factor
- UniProt
- P04275
- 物种
- Human
产品详情
- 靶点名称
- von Willebrand Factor
- 蛋白名称
- von Willebrand factor
- 蛋白别名
- von Willebrand factor; vWF) [Cleaved into: von Willebrand antigen 2; von Willebrand antigen II]
- 靶点基因名
- VWF
- 基因别名
- F8VWF
- UniProt
- P04275
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Von Willebrand factor (VWF) is a large multimeric glycoprotein essential for hemostasis, primarily facilitating platelet adhesion to sites of vascular injury. The protein's structure includes multiple binding domains that interact with platelets and collagen, critical for its hemostatic function. It exists in plasma as multimers ranging from 500 to 20,000 kDa, with larger forms being more functionally active. VWF plays a dual role by not only promoting platelet aggregation but also serving as a carrier for coagulation factor VIII, extending its half-life in circulation. Deficiencies or dysfunctions in VWF lead to von Willebrand disease (VWD), the most common inherited bleeding disorder, characterized by prolonged bleeding due to inadequate platelet function and reduced clot formation. Additionally, abnormal VWF levels can be associated with thrombotic conditions such as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), where excessive VWF leads to increased thrombosis risk.