antibody

Sheep A' Von Willebrand Factor

MBS315996

1 ml

405 USD

polyclonal

sheep

nonconjugated

Antibody

Sheep A' Von Willebrand Factor

Von Willebrand Factor Antigen

Sheep Antibody to Human Von Willebrand Factor (VWF)

von Willebrand factor preproprotein; von Willebrand factor; von Willebrand factor; OTTHUMP00000238431; coagulation factor VIII VWF; von Willebrand factor; von Willebrand antigen II

VWF; VWF; VWD; F8VWF; F8VWF

VWF_HUMAN

Polyclonal

Sheep

2813

Von Willebrand Factor Antigen. The unconjugated immunoglobulin gives no arcs when tested by IEP against human serum. Identity has been confirmed by double diffusion (Ouchterlony) against human VWF and an anti-human VWF of known specificity.

Delipidated and, if necessary, absorbed to monospecificity by use of solid-phase absorbants. Following adsorption an immunoglobulin fraction is produced by ion-exchange chromatography and preservatives are added. The immunoglobulin fraction is then conjug

FITC, Liquid

10mg/ml

Upon receipt, store at 2 to 8 degree C. Slight precipitation can occur upon storage. This should be removed by centrifugation and should not affect performance characteristics.

Evaluated by direct immunofluorescence on frozen sections of human tonsil and a dilution range of 1:50 to 1:100 is recommended for this techniques. Each laboratory should determine an optimum working titer for use in its particular application. Other appl

Immunogen: Human von Willebrand Factor Antigen (VWF): Prepared from citrated human plasma. >95% pure (SDS-PAGE)

Buffer: PBS, pH 7.2. Preservative: 0.099% Sodium azide. Important Note: Centrifuge before opening to ensure complete recovery of vial contents. Warnings: This product contains sodium azide, which has been classified as Xn (Harmful), in European Directive 67/548/EEC in the concentration range of 0.1 - 1.0%. When disposing of this reagent through lead or copper plumbing, flush with copious volumes of water to prevent azide build-up in drains.

Polyclonal Antibodies to Platelets and Hemostasis Antigens

89191868

NP_000543.2

NM_000552.3

P04275

309,265 Da

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]

Function: 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. Subunit structure: Multimeric. Interacts with F8. Ref.19 Ref.22. Subcellular location: Secreted. Secreted extracellular space extracellular matrix. Note: Localized to storage granules. Ref.19. Tissue specificity: Plasma. Domain: The von Willebrand antigen 2 is required for multimerization of vWF and for its targeting to storage granules. Post-translational modification: All cysteine residues are involved in intrachain or interchain disulfide bonds.N- and O-glycosylated. Ref.21 Ref.23 Ref.25. Involvement in disease: Defects in VWF are the cause of von Willebrand disease type 1 (VWD1) [. MIM:193400]. 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. Ref.59 Ref.60Defects in VWF are the cause of von Willebrand disease type 2 (VWD2) [. MIM:613554]. 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) [. MIM:277480]. 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. Sequence similarities: Contains 1 CTCK (C-terminal cystine knot-like) domain.Contains 4 TIL (trypsin inhibitory-like) domains.Contains 3 VWFA domains.Contains 3 VWFC domains.Contains 4 VWFD domains. Sequence caution: The sequence AAB59512.1 differs from that shown. Reason: Contaminating sequence. Sequence of unknown origin in the N-terminal part.

1 ml

405 USD