The product is a multimeric glycoprotein that consists of disulfide-bridge linked dimers. It is usually circulating in plasma as a stable complex with the coagulation factor VIII. The human vWF is a complex that also contains the coagulation factor VIII.
The vWF and Factor VIII complex is indicated for the prevention of excessive bleeding during and after minor and major surgery in adult and pediatric von Willebrand disease patients. It is also indicated for the on-demand treatment and control of bleeding episodes. The von Willebrand disease is an inherited disorder characterized by the deficiency or misfunction of the von Willebrand factor (vWF). Due to this deficiency, the blood cannot clot properly and the patients that present this disease are prone to prolonged or excessive bleeding. There are three types of this disease, and type 3 is an autosomal recessive inherited disorder marked by very low or absent levels of vWF.
Examples of Clinical Use:
Von Willebrand disease
The efficacy in clinical trials of vWF in surgical procedures is of 96.7% regardless the type of von Willebrand disease. In a perioperative setting, the hemostatic effects were rated as excellent in a dose of 848.6 IU/kg and no anti-factor VII or inhibitors were detected. These excellent results come from the re-establishment of platelet-adhesion to the subendothelium at sites of vascular damage and the aggregation of platelets. vWF allows the protection of factor VIII from the rapid degradation and thus it restores normal coagulation.
Mechanism of action:
The vWF is involved in primary and secondary homeostasis and it is also a carrier and stabilizing protein that protects the coagulation factor VIII from proteolysis and clearance. vWF also presents an adhesive function in which it mediates the binding between platelets and subendothelial tissues. The external human vWF acts as the endogenous vWF and thus, it presents all the functions abovementioned.
Target 1. Coagulation factor VIII; Target 2. Collagen alpha-1(I) chain; Enzyme 1. A disintegrin and metalloproteinase with thrombospondin motifs 13
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