catalog number :
MBS219591
products full name :
MOUSE ANTI HUMAN VON WILLEBRAND FACTOR
products short name :
VON WILLEBRAND FACTOR
other names :
von Willebrand factor preproprotein; von Willebrand factor; von Willebrand factor; coagulation factor VIII VWF; von Willebrand factor; von Willebrand antigen II
other gene names :
VWF; VWF; VWD; F8VWF; F8VWF; vWF
uniprot entry name :
VWF_HUMAN
form :
Purified. Purified IgG - liquid
concentration :
IgG concentration 1.0mg/ml
storage stability :
Store at 4 degree C or at -20 degree C if preferred. Storage in frost-free freezers is not recommended. This product should be stored undiluted. Avoid repeated freezing and thawing as this may denature the antibody. Should this product contain a precipitate we recommend microcentrifugation before use. Shelf Life: 18 months from date of despatch.
tested application :
ELISA (EIA), Immunohistology Paraffin*, Protein Purification (PP), Radioimmunoassays (RIA)
app notes :
Immunohistology - Paraffin: Minimum Dilution: 1/50; Maximum Dilution: 1/200; Application Note: This product requires protein digestion pre-treatment of paraffin sections e.g. trypsin or pronase.
other info1 :
Perservative Stabilisers: 0.09% Sodium Azide (NaN3) . Preparation: Purified IgG prepared by affinity chromatography on Protein G from tissue culture supernatant
other info2 :
Immunogen: Human Factor VIII complex partially purified from Factor VIII concentrate. Histology Positive Control Tissue: Human tonsil, thymus, liver, spleen or kidney. Buffer Solution: Phosphate buffered saline. Target Species: Human
products description :
Mouse anti Human von Willebrand factor antibody, clone RFF-VIII R/1 recognizes human von Willebrand factor (vWF), also known as Factor VIII related antigen, 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 (250kDa) 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. Mouse anti Human von Willebrand factor, 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 recognized is present only on the intact multimeric form of vWF and is abolished by mild denaturation with SDS. Mouse anti Human von Willebrand factor, clone RFF-VIII R/1 does not recognize human Factor VIII. Clone RF-VIII R/1 may be used as a capture antibody in immunoassays for vWF in combination with clone RFF-VIII R/2 as a detection reagent.
ncbi acc num :
NP_000543.2
ncbi gb acc num :
NM_000552.3
ncbi mol weight :
38,745 Da
ncbi pathways :
Blood Clotting Cascade Pathway 198840!!Complement And Coagulation Cascades Pathway 198880!!Complement And Coagulation Cascades Pathway 83073!!Complement And Coagulation Cascades Pathway 484!!ECM-receptor Interaction Pathway 83068!!ECM-receptor Interaction Pathway 479!!Focal Adhesion Pathway 198795!!Focal Adhesion Pathway 83067!!Focal Adhesion Pathway 478!!Formation Of Fibrin Clot (Clotting Cascade) Pathway 106057
ncbi summary :
The glycoprotein encoded by this gene functions as both an antihemophilic factor carrier and a platelet-vessel wall mediator in the blood coagulation system. It is crucial to the hemostasis process. Mutations in this gene or deficiencies in this protein result in von Willebrand's disease. An unprocessed pseudogene has been found on chromosome 22. [provided by RefSeq, Jul 2008]