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Description

von Willebrand factor (UniProt: P04275, also known as vWF) is encoded by the VWF (also known as F8VWF) gene (Gene ID:7450) in human. vWF is a multimeric plasma glycoprotein that is synthesized by endothelial cells and plays an important in the maintenance of hemostasis. It is synthesized with a signal peptide (aa 1-22), which is subsequently cleaved off in the mature form, which is then cleaved into von Willebrand antigen 2 (aa 23-763) and vWF (aa 764-2813). vWF is known to promote 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. vWF 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. Mutations in VWF gene are linked to von Willebrand diseases 1-3 that are common hemorrhagic disorders caused by impaired platelet aggregation. Two isoforms of vWF have been described that are produced by alternative splicing.