Human VWF / Von Willebrand Factor

Mouse

Human (tested or 100% immunogen sequence identity)

IgG1 Monoclonal

RFF-VIII R/2

Unconjugated

Protein G purified

Unmodified

Human Factor VIII complex partially purified from Factor VIII concentrate.

Recognizes human von Willebrand factor (vWF), also known as Factor VIII related antigen, a blood glycoprotein involved in blood coagulation. It stabilizes 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), characterized by varying degrees of susceptibility to bleeding. Symptoms might include nosebleeds, bleeding gums, easy bruising, menorrhagia or gastrointestinal bleeding. Various forms of vWD exist with differing severities, determined by the type of defect. Clone RF-VIII R/2 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 neutralize ristocetin-induced platelet aggregation, platelet binding to the subendothelium and ristocetin-induced binding of vWF to platelets. It also inhibits platelet adhesion to glass beads. The epitope recognized is present only on the intact multimeric form of vWF and is abolished by mild denaturation with SDS. The antibody does not recognize human Factor VIII. Clone RF-VIII R/2 may be used to detect vWF in immunoassays in combination with clone RFF-VIII R/1 as a capture reagent.

• IHC - Frozen (1:25 - 1:100)
• ELISA
• Purification
• Radioimmunoassay

• IHC - Paraffin
• Western blot

PBS, 0.09% Sodium Azide

Store at 4°C or at -20°C. Store undiluted. Avoid freeze-thaw cycles. Microcentrifugation recommended if solution contains precipitate.

For research use only. Intended for use by laboratory professionals.

This antibody carries the LSBio 100% Guarantee.

Uniprot: P04275 NCBI: NM_000552 NP_000543.2