catalog number :
MBS2880826
products type :
ELISA Kit
products full name :
Human von Willebrand factor ELISA Kit
products short name :
[von Willebrand factor]
products name syn :
[von Willebrand factor; vWF; VWF; F8VWF]
other names :
[von Willebrand factor preproprotein; 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; F8VWF; vWF]
uniprot entry name :
VWF_HUMAN
storage stability :
For long term storage, please store the entire kit at -20 degree C.
image1 heading :
Typical Testing Data/Standard Curve (for reference only)
other info1 :
Samples: Serum, Plasma, Tissue Homogenates, Cell Culture Supernates Or Other Biological Fluids.
products description :
Intended Uses: This immunoassay kit allows for the in vitro quantitative determination of target antigen concentrations in serum, plasma, tissue homogenates, cell culture supernates or other biological fluids. Principle of the Assay: The microtiter plate provided in this kit has been pre-coated with an antibody specific to target antigen. Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated antibody preparation specific for target antigen and then avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. Then a TMB substrate solution is added to each well. Only those wells that contain target antigen, biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The concentration of target antigen in the samples is then determined by comparing the O.D. of the samples to the standard curve.
ncbi acc num :
NP_000543.2
ncbi gb acc num :
NM_000552.3
ncbi mol weight :
38,745 Da
ncbi pathways :
ARMS-mediated Activation Pathway (1269471); Axon Guidance Pathway (1270303); Blood Clotting Cascade Pathway (198840); Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Cytokine Signaling In Immune System Pathway (1269310); DAP12 Interactions Pathway (1269283); DAP12 Signaling Pathway (1269284); Developmental Biology Pathway (1270302)
ncbi summary :
This gene encodes a glycoprotein involved in hemostasis. The encoded preproprotein is proteolytically processed following assembly into large multimeric complexes. These complexes function in the adhesion of platelets to sites of vascular injury and the transport of various proteins in the blood. Mutations in this gene result in von Willebrand disease, an inherited bleeding disorder. An unprocessed pseudogene has been found on chromosome 22. [provided by RefSeq, Oct 2015]
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: Secreted, signal peptide; Extracellular matrix; Secreted; Motility/polarity/chemotaxis; Cell adhesion. Chromosomal Location of Human Ortholog: 12p13.3. Cellular Component: endoplasmic reticulum; external side of plasma membrane; extracellular matrix; extracellular region; proteinaceous extracellular matrix. 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; blood coagulation, intrinsic pathway; cell adhesion; cell-substrate adhesion; extracellular matrix organization and biogenesis; hemostasis; liver development; placenta development; platelet activation; platelet degranulation; protein homooligomerization; response to wounding. Disease: Von Willebrand Disease, Type 1; Von Willebrand Disease, Type 2; Von Willebrand Disease, Type 3
size4 :
10x96-Strip-Wells