Von Willebrand Factor (VWF) is a glycoprotein encoded by the gene located on the short arm of chromosome 12. Under normal physiological conditions, VWF is mainly produced by endothelial cells and stored in Weibel-Palade bodies, while the VWF produced by megakaryocytes is stored in platelet granules. When there is an injury to the blood vessels, VWF acts as a bridge that enables platelets to adhere to collagen fibers, leading to the formation of a blood clot and achieving hemostasis. The concentration of VWF in normal human plasma is about 10mg/L. It can increase in response to endothelial cell stimulation or damage and during periods of physiological stress. Elevated levels of VWF are associated with an increased risk of thrombotic diseases, and certain mutations in the VWF gene can lead to von Willebrand disease (vWD), a bleeding disorder characterized by a deficiency or dysfunction of VWF.
中文
