免 疫 原：KLH conjugated synthetic peptide derived from human Von Willebrand Factor
抗原表位：351-450/2813
SWISS：P04275
Gene ID ：7450
Human Gene ID：7450

Von Willebrand Factor (VWF) was previously known as Factor VIII related antigen. VWF is synthesized exclusively by endothelial cells and megakaryocytes, and stored in the intracellular granules or constitutively secreted into plasma. This glycoprotein functions as both an antihemophilic factor carrier and a platelet vessel wall mediator in the blood coagulation system. Important in the maintenance of homeostasis, it participates in platelet vessel wall interactions by forming a noncovalent complex with coagulation factor VIII at the site of vascular injury. The Von Willebrand factor has functional binding domains to platelet glycoprotein Ib, glycoprotein IIb/IIIa, collagen and heparin. Mutations in this gene or deficiencies in this protein result in Von Willebrand's disease. VWD is characterized by frequent bleeding (gingival, minor skin quantitative lacerations, menorrhagia, etc.).
Function：Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex GPIb-IX-V. Also acts as a chaperone f
Subunit：Multimeric. Interacts with F8.
Subcellular Location：Secreted. Secreted, extracellular space, extracellular matrix. Note=Localized to storage granules.
Tissue Specificity：Plasma.
Post-translational modifications：All cysteine residues are involved in intrachain or interchain disulfide bonds.
N- and O-glycosylated.
DISEASE：Defects in VWF are the cause of von Willebrand disease type 1 (VWD1) [MIM:193400]. A common hemorrhagic disorder due to defects in von Willebrand factor protein and resulting in impaired platelet aggregation. Von Willebrand disease type 1 is characterized
Similarity：Contains 1 CTCK (C-terminal cystine knot-like) domain.
Contains 4 TIL (trypsin inhibitory-like) domains.
Contains 3 VWFA domains.
Contains 3 VWFC domains.
Contains 4 VWFD domains.
Important Note：This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.