Anti-Human von Willebrand Factor Antibody Pair

Product Description: 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. Protein Function: Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex GPIb-IX-V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma [The Uniprot Consortium]