antibody

Rabbit anti-human von Willebrand factor polyclonal Antibody, HRP conjugated

MBS1490244

0.05 mg

160 USD

polyclonal

rabbit

HRP

ELISA, enzyme immunoassay

Antibody

Rabbit anti-human von Willebrand factor polyclonal Antibody, HRP conjugated

[von Willebrand factor]

[vWF; von Willebrand antigen II; F8VWF]

[von Willebrand factor preproprotein; von Willebrand factor; von Willebrand factor; von Willebrand factor; von Willebrand antigen 2Alternative name(s):von Willebrand antigen II]

[VWF]

[VWF; VWF; VWD; F8VWF; F8VWF; vWF]

VWF_HUMAN

Polyclonal

IgG

Rabbit

Human

2813

Caprylic Acid Ammonium Sulfate Precipitation Purified

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.

ELISA (EIA)

Storage Buffer: Preservative: 0.03% Proclin 300. Constituents: 50% Glycerol, 0.01M PBS, PH 7.4. Conjugate: HRP

Immunogen: Recombinant human von Willebrand factor protein. Santa Cruz Alternative: Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-8068 / sc-14014

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.

89191868

NP_000543.2

NM_000552.3

P04275

38,745 Da

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)

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]

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: Motility/polarity/chemotaxis; Cell adhesion; Extracellular matrix; Secreted, signal peptide; Secreted. 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

0.05 mg

160 USD

0.1 mg

235