Von Willebrand disease is a congenital bleeding disorder caused by defects in the von Willebrand factor protein (VWF).
VWF is a multimeric glycoprotein that is found in endothelial cells, plasma and platelets, and it is involved in the coagulation of blood at injury sites.
VWF acts as a carrier protein for Factor VIII, a cofactor required for coagulation, and it promotes platelet adhesion and aggregation.
Several factors are known to stimulate the binding of VWF to platelets, including glycoprotein 1b, ristocetin, botrocetin, collagen, sulphatides and heparin.
Of the several domains contained within VWF, the A1, A2 and A3 domains have been shown to mediate this activation.
VWF is thought to undergo a variety of posttranslational modifications that influence the affinity and availability for Factor VII, including cleavage of the propeptide and formation of N-terminal intersubunit disulfide bonds.
Applications:Suitable for use in Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：50ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity： Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.3, 0.05% sodium azide.
Specificity：Recognizes human VWF. Species Crossreactivity: mouse and rat
Isotype：IgG
Calc Applications Abbrev：IHC
Calc Crossreactivity：Hu Mo Rt
Immunogen：Recombinant protein corresponding full length human VWF.
