Human von Willebrand factor (vWF), also known as Factor VIII related antigen, is a blood glycoprotein involved in blood coagulation. It stabilises circulating Factor VIII by binding to it and protecting it from cleavage and delivers it to sites of vascular injury. vWF also promotes the adhesion of platelets to sites of vascular damage by forming a molecular bridge between collagen on exposed endothelial cells and the GPIb binding sites of platelets circulating in the blood. vWF circulates in the blood as large multimers, with each monomer (250kD) containing a number of specific domains. Hereditary or acquired defects in vWF lead to von Willebrand disease (vWD), characterised by varying degrees of susceptibility to bleeding. Symptoms might include nosebleeds, bleeding gums, easy bruising, menorrrhagia or gastrointestinal bleeding. Various forms of vWD exist with differing severities, determined by the type of defect.
Clone RFF-VIII R/1 has a high affinity for an epitope within the platelet GPIb-binding site that is responsible for biological activity. As such the antibody is a potent inhibitor of vWF activity. It can completely neutralise ristocetin-induced platelet aggregation and ristocetin-induced binding of vWF to platelets. It also inhibits platelet adhesion to glass beads. The epitope recognised is present only on the intact multimeric form of vWF and is abolished by mild denaturation with SDS. The antibody does not recognise human Factor VIII.
Applications
Suitable for use in RIA, ELISA, Immunohistochemistry and Protein Purification. Other applications not tested.
Recommended Dilution
Immunohistochemistry (Paraffin): 1:50-1:200. Requires antigen retrieval using pronase digestion prior to staining of paraffin sections. Optimal dilutions to be determined by the researcher.
IHC Positive Control Tissues
Human tonsil, thymus, liver, spleen or kidney.
ELISA Matched Pair
Capture Antibody: Cat. #V2700-01J Detection Antibody: Cat. #215209
Recommended Secondary Antibodies
I1904-06F IgG, H&L (FITC) (X-Adsorbed)(rat adsorbed) Pab GtxMo I1904-06H IgG, H&L (X-Adsorbed) (HRP)(rat adsorbed) Pab GtxMo I1904-09W IgG, H&L, X-adsorbed (HRP) (rat adsorbed) Pab GtxMo I1904-65N IgG (HRP) Pab Rb xMo I1903-07E IgG (HRP) Pab Rb xMo
Storage and Stability
May be stored at 4°C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20°C. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Immunogen
Human Factor VIII complex partially purified from Factor VIII concentrate.
Form
Supplied as a liquid in PBS, pH 7.4, 0.09% sodium azide.
Purity
Purified from culture superantant by Protein A affinity chromatography.
Specificity
Recognizes human von Willebrand factor. Recognizes an epitope within the platelet GPIb-binding site. Does not recognize human Factor VIII.
TDS
NCBI
UniProt
Tech Support
Customer Service
