Molecular weight (DA)
309kD
Immunogen
Synthesized peptide derived from part region of human protein AA range: 911-960
Specificity
VWF Polyclonal Antibody detects endogenous levels of protein.
Formulation
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
Dilution rate
IHC-p 1:50-300. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
This gene encodes a glycoprotein involved in hemostasis. The encoded preproprotein is proteolytically processed following assembly into large multimeric complexes. These complexes function in the adhesion of platelets to sites of vascular injury and the transport of various proteins in the blood. Mutations in this gene result in von Willebrand disease, an inherited bleeding disorder. An unprocessed pseudogene has been found on chromosome 22. [provided by RefSeq, Oct 2015],
Function
disease:Defects in VWF are associated with various forms of von Willebrand disease (VWD) [MIM:193400, 277480]. VWD is characterized by frequent bleeding (gingival, minor skin quantitative lacerations, menorrhagia, etc.). 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. There are subtypes (A to H) of type II VWD; for example: type IIA is characterized by the absence of VWF high molecular weight multimers in plasma.,domain:The von Willebrand antigen 2 is required for multimerization of vWF and for its targeting to storage granules.,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 for coagu
Protein name
von Willebrand factor (vWF) [Cleaved into: von Willebrand antigen 2 (von Willebrand antigen II)]
Fields
>>PI3K-Akt signaling pathway;>>Focal adhesion;>>ECM-receptor interaction;>>Complement and coagulation cascades;>>Platelet activation;>>Neutrophil extracellular trap formation;>>Human papillomavirus infection;>>Coronavirus disease - COVID-19
Human protein sequence Database
P04275
Mouse protein sequence database
Q8CIZ8
Rat protein sequence database
Q62935
Cellular localization
Secreted . Secreted, extracellular space, extracellular matrix . Localized to storage granules.
Tissue expression
Plasma.
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