Von Willebrand factor

Von Willebrand factor (vWF) is a blood plasma protein that is critical for blood clotting (coagulation). It is named after Erik Adolf von Willebrand, a Finnish physician who first described the condition Von Willebrand disease in 1926.

File:VWF-GP1ba.png

VWF-GP1ba

Function

vWF is produced in endothelial cells and megakaryocytes. It circulates in the blood plasma, binding to other proteins, particularly factor VIII, and helps them to function effectively. Its primary role is to initiate the process of platelet adhesion to the site of a blood vessel injury, which is the first step in the formation of a blood clot.

Structure

vWF is a large multimeric glycoprotein. The monomeric unit is a 2050 amino acid protein with a molecular weight of about 250 kDa. These monomers link together to form multimers that can be over 20,000 kDa in size.

Clinical significance

Deficiency or dysfunction of vWF leads to Von Willebrand disease, the most common inherited bleeding disorder. This disease is characterized by prolonged bleeding time, which can lead to significant clinical problems. There are three types of Von Willebrand disease, each with different genetic and clinical features.

Research

Research into vWF has increased our understanding of the process of blood clotting and has led to the development of new treatments for bleeding disorders. It is also being investigated for its potential role in other diseases, such as cardiovascular disease and cancer.

See also

• Blood clotting
• Von Willebrand disease
• Factor VIII
• Endothelial cells
• Megakaryocytes

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