Von Willebrand Factor Recombinant Rabbit Monoclonal Antibody [JJ09-34]

功能

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.

背景文献

1. Nurnberg ST et al. Coronary Artery Disease Associated Transcription Factor TCF21 Regulates Smooth Muscle Precursor Cells That Contribute to the Fibrous Cap. PLoS Genet 11:e1005155 (2015).

2. Cheung K et al. CD31 signals confer immune privilege to the vascular endothelium. Proc Natl Acad Sci U S A 112:E5815-24 (2015).

组织特异性

Plasma.

翻译后修饰

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

亚细胞定位

Secreted.

UNIPROT

SwissProt: P04275 Human

SwissProt: Q8CIZ8 Mouse

别名

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