catalog number :
MBS9388169
products full name :
VWF Antibody
products short name :
[VWF]
other names :
[VWF; von Willebrand factor; von Willebrand factor; Von Willebrand factor; von Willebrand antigen 2Alternative name(s):von Willebrand antigen II]
products gene name :
[VWF]
other gene names :
[Vwf; Vwf; VWD; F8VWF; AI551257; C630030D09; 6820430P06Rik; B130011O06Rik; vWF]
reactivity :
Human, Mouse, Rat
specificity :
von Willebrand factor (VWF)
purity :
The antiserum was purified by peptide affinitychromatography using SulfoLink™ Coupling Resin (ThermoFisher Scientific).
storage stability :
Store at 4°C.
tested application :
ELISA (EIA), Immunohistochemistry (IHC)
app notes :
ELISA: (1:1,000~100,000). Immunohistochemistry: (1:50~200). For western blot, incubate membrane with diluted Ab in 5%w/v milk , 1X TBS, 0.1% Tween(R)20 at 4°C with gentle shaking, overnight.
image1 heading :
Immunohistochemistry (IHC)
other info1 :
Immunogen: Purified Rabbit Anti Human VWF Polyclonal antibody.
other info2 :
Subcellular Location: Secreted. Secreted > extracellular space > extracellularmatrix. Localized to storage granules. Tissue Specificity: Plasma. Similarity: The von Willebrand antigen 2 is required for multimerizationof vWF and for its targeting to storage granules. Buffer: PBS with 0.02% Sodium Azide, 2%BSA,PH7.2.
products description :
Important in the maintenance of hemostasis, it promotesadhesion of platelets to the sites of vascular injury byforming a molecular bridge between sub-endothelialcollagen matrix and platelet-surface receptor complex GPIb-IX-V.
ncbi acc num :
ABC86573.1
ncbi gb acc num :
NM_000552.3
ncbi pathways :
Blood Clotting Cascade Pathway (198388); Complement And Coagulation Cascades Pathway (198335); Complement And Coagulation Cascades Pathway (83270); Complement And Coagulation Cascades Pathway (484); ECM-receptor Interaction Pathway (83265); ECM-receptor Interaction Pathway (479); Focal Adhesion Pathway (198353); Focal Adhesion Pathway (83264); Focal Adhesion Pathway (478); Formation Of Fibrin Clot (Clotting Cascade) Pathway (1110054)
uniprot summary :
VWF: Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex GPIb-IX-V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma. Defects in VWF are the cause of von Willebrand disease type 1 (VWD1). A common hemorrhagic disorder due to defects in von Willebrand factor protein and resulting in impaired platelet aggregation. Von Willebrand disease type 1 is characterized by partial quantitative deficiency of circulating von Willebrand factor, that is otherwise structurally and functionally normal. Clinical manifestations are mucocutaneous bleeding, such as epistaxis and menorrhagia, and prolonged bleeding after surgery or trauma. Defects in VWF are the cause of von Willebrand disease type 2 (VWD2). A hemorrhagic disorder due to defects in von Willebrand factor protein and resulting in impaired platelet aggregation. Von Willebrand disease type 2 is characterized by qualitative deficiency and functional anomalies of von Willebrand factor. It is divided in different subtypes including 2A, 2B, 2M and 2N (Normandy variant). The mutant VWF protein in types 2A, 2B and 2M are defective in their platelet- dependent function, whereas the mutant protein in type 2N is defective in its ability to bind factor VIII. Clinical manifestations are mucocutaneous bleeding, such as epistaxis and menorrhagia, and prolonged bleeding after surgery or trauma. Defects in VWF are the cause of von Willebrand disease type 3 (VWD3). A severe hemorrhagic disorder due to a total or near total absence of von Willebrand factor in the plasma and cellular compartments, also leading to a profound deficiency of plasmatic factor VIII. Bleeding usually starts in infancy and can include epistaxis, recurrent mucocutaneous bleeding, excessive bleeding after minor trauma, and hemarthroses. Protein type: Cell adhesion; Extracellular matrix; Motility/polarity/chemotaxis; Secreted; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 6 F3 6 59.32 cM. Cellular Component: cytoplasm; endoplasmic reticulum; external side of plasma membrane; extracellular matrix; extracellular region; extracellular space. Molecular Function: chaperone binding; collagen binding; glycoprotein binding; identical protein binding; immunoglobulin binding; integrin binding; protease binding; protein binding; protein homodimerization activity; protein N-terminus binding. Biological Process: blood coagulation; cell adhesion; cell-substrate adhesion; hemostasis; liver development; placenta development; platelet activation; protein homooligomerization