Anti-Von Willebrand Factor Antibody [JJ09-34]

HUABIO

Anti-Von Willebrand Factor Antibody [JJ09-34]

7

Western Blot

WB,IHC-P

Research Use Only

Monoclonal

JJ09-34

1 mg/ml

Unconjugated

VWF

von Willebrand factor

F8VWF, VWD, von Willebrand factor, Factor VIII related antigen, coagulation factor VIII VWF

Rabbit

IgG

von Willebrand factor

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.

Human

Human

-20°C,2°C to 8°C

41116161