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).
Categories
Primary Antibodies
Cellular Localization
Extracellular matrix, Secreted
Clonality
polyclonal
Description
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 (residues 56-256 aa) of pre-pro-vWF recognizes von Willebrand antigen 2 (75-83 kDa) and pro-vWF (309-320 kDa). (PMID: 9759493; 12067899; 22452980; 8874191)
Host
Rabbit
Immunogen
von Willebrand factor
Isotype
IgG
Molecular Weight
75 kDa,309 kDa
Reactivity
Human, Mouse, Rat
Regulatory
RUO
Subcellular Location
Extracellular matrix, Secreted
Synonyms
F8VWF, von Willebrand antigen II, von Willebrand factor, VWD, VWF
Uniprot
P04275
Research Area
Immunology, Cardiovascular
Weight
309kDa,observed 70kDa,309kDa
Form
liquid
Format
liquid
Purification
Immunogen affinity purified
Purity
>=95% as determined by SDS-PAGE
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20°C for 12 months(Avoid repeated freeze / thaw cycles.)