Von Willebrand Factor Recombinant Rabbit Monoclonal Antibody [JJ09-34] (ET1701-87)

UNIPROT #

P04275

PROTEIN NAME

Von Willebrand Factor

SYNONYMS

Coagulation factor VIII antibody;Coagulation factor VIII VWF antibody;F8VWF antibody;Factor VIII related antigen antibody;von Willebrand antigen 2 antibody;von Willebrand antigen II antibody;Von Willebrand disease antibody;VWD antibody;vWF antibody;VWF_HUMAN antibody

TISSUE SPECIFICITY

Plasma.

POST-TRANSLATIONAL MODIFICATION

All cysteine residues are involved in intrachain or interchain disulfide bonds.; N- and O-glycosylated.

SUBCELLULAR LOCATION

Secreted.

FUNCTION

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.