VWF, VWFpp antibody

Von Willebrand factor(vWF) is a large multimeric glycoprotein that is crucial to the hemostasis process. It supports platelet adhesion and carries factor VIII(FVIII). Deficiency of vWF results in von Willebrand disease(vWD), a common inherited bleeding disorder. The pre-pro-vWF is comprised of 2,813 amino acids(aa) which encompass a 22-aa signal peptide, a 741-aa large propeptide(vWF propeptide, vWFpp, also called von Willebrand antigen II) and 2,050 aa making up the mature vWF. The von Willebrand antigen 2 is required for multimerization of vWF and for its targeting to storage granules. This antibody raised against the N-terminal region of pre-pro-vWF recognizes von Willebrand antigen 2(75-83 kDa) and pro-vWF(309-320 kDa).