C16572

CTRP

von Willebrand factor (2813 aa, ~309 kDa) is encoded by the human VWF gene. This protein is involved in platelet adhesion and blood coagulation.

Human von Willebrand factor, a 270 kD multimeric plasma glycoprotein, has functional binding domains to platelet glycoprotein Ib, glycoprotein Iib/IIIa, collagen and heparin. It mediates platelet adhesion to injured vessel walls and serves as a carrier and stabiliser for coagulation factor VIII. It is expressed in endothelial cells, platelets, megakaryocytes and a number of tumors.

Important in the maintenance of homeostasis, multimeric Factor VIII Related Antigen participates in platelet-vessel wall interactions by forming a non-covalent complex with coagulation factor VIII at sites of vascular injury, creating a surface to which platelets adhere. Plasma factor VIII related protein is a large glycoprotein component, synthesized by endothelial cells and megakaryocytes, that circulates in the plasma complexed with factor VIII. VWF contains three A domains, three C domains, four D domains, and one C-terminal cysteine knot-like domain. All cysteine residues of von Willebrand factor are involved in intrachain or interchain disulfide bonds. Defects in F8VWF are associated with various forms (types I-III) of von Willebrand disease (VWD). There are subtypes of type II VWD (A to H); type IIA is characterized by the absence of F8VWF multimers in plasma.

von Willebrand Factor

von Willebrand Factor

von_Willebrand_Factor

CL1770518

613160

von Willebrand Factor

NCIT:C16572

von Willebrand Factor

Amino Acid, Peptide, or Protein

P04275