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Tufts OCW material is licensed under a Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported License. This is a good idea, because the main job of von Willebrand factor is to act as the glue that sticks the platelets down onto the subendothelium! This means that patients have a hard time gluing platelets to their subendothelial proteins during clot formation (so the initial platelet plug doesn’t form as well). Von Willebrand Factor is a huge multimeric protein that is made by megakaryocytes and endothelial cells.


They also may have a hard time forming fibrin, since von Willebrand factor is a carrier (degradation-preventing) molecule for factor VIII, and factor VIII is a critical factor in the coagulation cascade. Endothelial cells synthesize von Willebrand factor and store it in little organelles called Weibel-Palade bodies (named after the two scientists who discovered them). Megakaryocytes (big cells in the bone marrow that give rise to platelets) synthesize von Willebrand factor, and it gets stored in the alpha granules of platelets.
It functions in both the initial, platelet-plug phase of hemostasis (in which it glues the platelets to the endothelium), and in the second, fibrin-forming phase of hemostasis (in which it serves as a carrier molecule for factor VIII that keeps factor VIII from being prematurely degraded).


The main things found in Weibel-Palade bodies are von Willebrand factor (described above) and P-selectin (a cell-adhesion molecule that endothelial cells use to catch passing leukocytes, allowing them to leave the blood vessel and migrate into the surrounding tissue).



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