catalog number :
MBS2007285
products full name :
Polyclonal Antibody to Von Willebrand Factor (vWF)
products short name :
Von Willebrand Factor (vWF)
other names :
von Willebrand factor preproprotein; von Willebrand factor; von Willebrand factor; coagulation factor VIII VWF; von Willebrand factor; von Willebrand antigen II
other gene names :
VWF; VWF; VWD; F8VWF; F8VWF; vWF
uniprot entry name :
VWF_HUMAN
sequence :
The target protein is fused with N-terminal His-Tag and its sequence is listed below. MGHHHHHHSGSEF-DVV FVLEGSDEVG EANFNKSKEF VEEVIQRMDV SPDATRISVL QYSYTVTMEY AFNGAQSKEE VLRHVREIRY QGGNRTNTGQ ALQYLSEHSF SPSQGDRVEA PNLVYMVTGN PASDEIKRLP GDIQVVPIGV GPHANMQELE RISRPIAPIF IRDFETLPRE APDLV
specificity :
The antibody is a rabbit polyclonal antibody raised against vWF. It has been selected for its ability to recognize vWF in immunohistochemical staining andwestern blotting.
purity :
Antigen-specific Affinity Chromatography.
form :
Supplied as solution form in PBS, pH7.4, containing 0.02% NaN3,50% glycerol.
storage stability :
Storage: Avoid repeated freeze/thaw cycles. Store at 4 degree C for frequent use. Aliquot and store at -20 degree C for 12 months. Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation and precipitation were observed.The loss rate is less than 5% within the expiration date under appropriate storage condition.
tested application :
WB; ICC; IHC-P; IHC-F; ELISA; IP; IF; FCM.
app notes :
Western blotting: 0.5-2ug/ml. Immunocytochemistry in formalin fixed cells: 5-20ug/ml. Immunohistochemistry in formalin fixed frozen section: 5-20ug/ml. Immunohistochemistry in paraffin section: 5-20ug/ml. Enzyme-linked Immunosorbent Assay: 0.05-2ug/ml. Optimal working dilutions must be determined by end user.
other info1 :
Source: Polyclonal antibody preparation. Traits: Liquid. Immunogen: Recombinant vWF (Asp1498~Val1665) expressed in E.coli.
ncbi acc num :
NP_000543.2
ncbi gb acc num :
NM_000552.3
ncbi mol weight :
309,265 Da
ncbi pathways :
Blood Clotting Cascade Pathway (198840); Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); ECM-receptor Interaction Pathway (83068); ECM-receptor Interaction Pathway (479); Focal Adhesion Pathway (198795); Focal Adhesion Pathway (83067); Focal Adhesion Pathway (478); Formation Of Fibrin Clot (Clotting Cascade) Pathway (106057)
ncbi summary :
The glycoprotein encoded by this gene functions as both an antihemophilic factor carrier and a platelet-vessel wall mediator in the blood coagulation system. It is crucial to the hemostasis process. Mutations in this gene or deficiencies in this protein result in von Willebrand's disease. An unprocessed pseudogene has been found on chromosome 22. [provided by RefSeq, Jul 2008]
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: Extracellular matrix; Secreted; Motility/polarity/chemotaxis; Secreted, signal peptide; Cell adhesion. Chromosomal Location of Human Ortholog: 12p13.3. Cellular Component: extracellular matrix; proteinaceous extracellular matrix; endoplasmic reticulum; extracellular region; external side of plasma membrane. Molecular Function: integrin binding; collagen binding; identical protein binding; protein binding; protein homodimerization activity; protease binding; chaperone binding; immunoglobulin binding; protein N-terminus binding; glycoprotein binding. Biological Process: platelet activation; extracellular matrix organization and biogenesis; platelet degranulation; hemostasis; response to wounding; blood coagulation; liver development; cell adhesion; blood coagulation, intrinsic pathway; protein homooligomerization; cell-substrate adhesion; placenta development. Disease: Von Willebrand Disease, Type 3; Von Willebrand Disease, Type 1; Von Willebrand Disease, Type 2