抗体

兔抗人类血管假性血友病因子, VWF多克隆抗体

MBS712337

0.05毫升

375美元

多克隆

兔

未共轭

人类, 小鼠

免疫印迹, 酶联免疫吸附测定, 免疫组化, 酶免疫法

抗体

兔抗人类血管假性血友病因子, VWF多克隆抗体

血管假性血友病因子, VWF

血管假性血友病因子;VWF;F8VWF;VWD

von Willebrand factor preproprotein; von Willebrand factor; von Willebrand factor; coagulation factor VIII VWF; von Willebrand factor; von Willebrand antigen II

VWF;VWF;VWD;F8VWF;F8VWF;vWF

VWF_HUMAN

多克隆

IgG

兔

Human,mouse,rat?Other species are not tested. Please decide the specificity by homology.

2813

抗原亲和纯化

液体

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

酶联免疫吸附测定(EIA), 免疫印迹(免疫印迹), 免疫组化(免疫组化)

ELISA: Use at an assay dependent dilution. WB : 1:200-1:2000. IHC : 1:20-1:200 (Recommender dilutions). Positive WB detected in: Jurkat cells, mouse spleen tissue . Positive IHC detected in: Human liver tissue, human brain tissue, human epididymis tissue, human heart tissue, human lung tissue, human ovary tissue, human placenta tissue, human skin tissue, human spleen tissue.

Immunogen: Human VWF. Conjugate : Non-conjugated

Storage Buffer: PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3.

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

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)

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]

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; Secreted; Secreted, signal peptide; Cell adhesion; Extracellular matrix. 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; protein N-terminus binding; immunoglobulin binding; glycoprotein binding. Biological Process: platelet activation; extracellular matrix organization and biogenesis; platelet degranulation; hemostasis; response to wounding; liver development; cell adhesion; blood coagulation; blood coagulation, intrinsic pathway; cell-substrate adhesion; protein homooligomerization; placenta development. Disease: Von Willebrand Disease, Type 3; Von Willebrand Disease, Type 1; Von Willebrand Disease, Type 2

0.05毫升

375美元

0.15毫升

715