Synonyms: Anti-vWF antibody produced in rabbit; Anti-von Willebrand factor precursor antibody produced in rabbit
MDL Number: MFCD00162556
Storage: -20C
Application: Anti-VWF antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org). Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Biochem Physiol Actions: Von Willebrand factor (VWF) is a large, adhesive glycoprotein synthesized only by endothelial cells and megakaryocytes, a platelet precursor. It is stored in a-granules in megakaryocytes or Weibel-Palade bodies in endothelial cells. In immature state, it is called as pre-pro-VWF molecule. It consists of D1 and D2 domains which are essential for multimerization. Upon maturation, it develops two other domains. During maturation the signal peptide of pro-VWF is cleaved to form C-terminal dimers in endoplasmic reticulum. The dimers are subjected to further modifications such as carbohydrate processing, sulfation and amino-terminal multimerization. The mature VWF plays major role in hemostasis. Firstly, it helps in attaching platelets through the glycoprotein Ib receptor to subendothelial tissue at the site of vascular injury. It also act as carrier protein for protecting coagulation factor VIII from proteolytic degradation by plasma enzymes. Deficiency or any alteration in VWF results in von Willebrand disease, a common hereditary bleeding disorder.
Legal Information: Prestige Antibodies is a registered trademark of Sigma-Aldrich Co. LLC