Von Willebrand Factor (VWF) Antibody – Goat Polyclonal

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, glycoprotein Ibalpha/IX/V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma

Subunit structure: Multimeric. Interacts with F8. Ref.2

Subcellular location: Secreted. Secreted › extracellular space › extracellular matrix. Note: Localized to storage granules. Ref.2

Tissue specificity: Plasma.

Domain: The propeptide is required for multimerization of vWF and for its targeting to storage granules.

Post-translational modification: All cysteine residues are involved in intrachain or interchain disulfide bonds

By similarity.N- and O-glycosylated

Involvement in disease: Note=Defects in VWF are the cause of von Willebrand disease (VWD) in the Scottish Terrier. VWD is characterized by frequent bleeding. Type I VWD is associated with a deficiency of VWF; type II by normal to decreased plasma level of VWF; type III by a virtual absence of VWF. Ref.4

Sequence similarities: 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.