Von Willebrand factor (VWF) is a blood glycoprotein required for normal hemostasis. VWF mediates the adhesion of platelets to sites of vascular damage by binding to specific platelet membrane glycoproteins and to constituents of exposed connective tissue. It also induces expression of the fibrinogen receptor and thus initiates platelet-platelet interaction. It is a carrier protein for blood clotting factor VIII and also stabilizes factor VIII and may serve to transport it to sites of vessel injury. It is assembled from identical approximately 250kD subunits into disulfide-linked multimers that may be greater 20,000kD. Mutations in VWD disrupt the complex biosynthetic process at several steps to impair the assembly, intracellular targeting, or secretion of VWF multimers. Deficiency of VWF leads to von Willebrand disease (VWD), and a large number of other diseases, including thrombotic thrombocytopenic purpura, Heyde's syndrome, and possibly hemolytic-uremic syndrome. It is mapped on human chromosome 12p13.3.
Applications:
Suitable for use in Immunohistochemistry. Other applications not tested.
Recommended Dilution:
Immunohistochemistry (formalin fixed paraffin embedded): 1:15-1:30
Optimal dilutions to be determined by the researcher.
Positive Control:
Tonsil
Storage and Stability:
May be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
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