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- 详细信息
- 文献和实验
- 技术资料
- 保质期:
3年
- 英文名:
human von Willebrand Factor – FVIII Free
- 保存条件:
-70度
- 库存:
大量
- 供应商:
Sekisui Diagnostics
- 规格:
100 μg
Description
Von Willebrand Factor (vWF) is a multimeric glycoprotein that circulates in plasma at a concentration of approximately 10 g/mL, composed of 260,000 Da subunits held together by disulfide bonds. vWF exists in plasma in various forms, from as small as a dimer with a Mr equal to 520,000 Da to large multimers with Mr equal to 10,000,000 Da.
vWF plays a dual role in normal hemostasis. One role is to facilitate platelet plug formation by forming a bridge between Platelet Glycoprotein 1b and exposed collagen in the sub endothelium. Its second function is to act as a stabilizing cofactor for Factor VIII (FVIII), binding to and delivering functional FVIII. FVIII binds to the amino terminal portion of vWF subunit in a 1:1 molar ratio. Von Willebrand Disease (vWD) is characterized by a decrease in the ability for this bridge to be formed, or as a decrease in biological activity as a result of either a vWF protein deficiency or the presence of abnormal vWF protein.
vWF is purified from citrated human plasma. This preparation is free of FVIII.
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文献和实验Multimeric Analysis of von Willebrand Factor
Von Willebrand Factor (vWF) in normal plasma is composed of a series of high molecular multimers, ranging in size from 8�105 to over 15�106 Daltons (1 ). The multimeric structure of vWF was first investigated by two-dimensional crossed
Screening for Candidate Mutations Causing von Willebrands Disease (vWD)
von Willebrand factor (vWF) is a large, complex glycoprotein that exists in plasma and platelets, and is synthesized by megakaryocytes and endothelial cells. vWF plays an essential role in hemostasis in at least two ways. It is involved
-Duistermaat j.j,et.al. Familial clustering of factor Ⅷ and von Willebrand Factor levels , Thromb Haemost 1998,79:323-7 2.O , Donnell J,Mumford A.D,Manning R,A,Elevation of F Ⅷ :C in venous thrombembolism is persistent and independent of the Axute









