Mouse anti-Human von Willebrand Factor Monoclonal Antibody | anti-vWD antibody

von Willebrand Factor (Factor VIII Related-Ag, Coagulation Factor VIII, Factor VIII Related Antigen, F8VWF, von Willebrand Antigen 2, von Willebrand Disease (vWD)) (HRP)

Cat. Num. AAA6123830

• Gene Names: VWF; VWD; F8VWF
• Reactivity: Human
• Applications: ELISA, Immunohistochemistry, Immunoprecipitation, Western Blot
• Purity: Purified by Protein A/G Affinity Chromatography.
• Synonyms: von Willebrand Factor; Monoclonal Antibody; von Willebrand Factor (Factor VIII Related-Ag; Coagulation Factor VIII; Factor VIII Related Antigen; F8VWF; von Willebrand Antigen 2; von Willebrand Disease (vWD)) (HRP); Factor VIII Related-Ag; von Willebrand Disease (vWD); anti-vWD antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG1,k
• Clone Number: VWF635
• Specificity: Recognizes human von Willebrand Factor (Factor VIII Related-Ag).
• Purity/Purification: Purified by Protein A/G Affinity Chromatography.
• Form/Format: Supplied as a liquid in 10mM PBS. No preservative added. BSA-Free. Labeled with horseradish peroxidase (HRP).

• Applicable Applications for anti-vWD antibody: ELISA (EIA), Immunohistochemistry (IHC) Paraffin, Immunoprecipitation (IP), Western Blot (WB)
• Application Notes: IHC-P: Requires boiling tissue sections in 10mM Citrate Buffer, pH 6.0, for 10-20 min followed by cooling at RT for 20 minutes; Applications are based on unconjugated antibody.
• Immunogen: Recombinant protein corresponding to human von Willebrand Factor fragment. Cellular Localization: Cytoplasmic
• Conjugate: HRP
• Positive Control: HUVEC cells or Tonsil
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Immunohistochemistry (IHC)

(Immunohistochemistry in formalin-fixed, paraffin-embedded human tonsil stained with)

Immunohistochemistry (IHC)

(Immunohistochemistry in formalin-fixed, paraffin-embedded human bone marrow stained with.)

Product Categories/Family for anti-vWD antibody

Antibodies; Coagulation Factors

References

1. Motta, A. et al. 2009. J Biomater Sci Polym Ed. 20: 1875-1897. 2. Germann, B. et al. 2008. Pharmazie. 63: 303-307.

NCBI and Uniprot Product Information

• NCBI GI #: 89191868
• NCBI GeneID: 7450
• NCBI Accession #: NP_000543.2
• NCBI GenBank Nucleotide #: NM_000552.3
• UniProt Accession #: P04275; Q99806
• Molecular Weight: 309,265 Da
• NCBI Official Full Name: von Willebrand factor preproprotein
• NCBI Official Synonym Full Names: von Willebrand factor
• NCBI Official Symbol: VWF
• NCBI Official Synonym Symbols: VWD; F8VWF
• NCBI Protein Information: von Willebrand factor; coagulation factor VIII VWF
• UniProt Protein Name: von Willebrand factor
• Protein Family: von Willebrand factor
• UniProt Gene Name: VWF
• UniProt Synonym Gene Names: F8VWF; vWF
• UniProt Entry Name: VWF_HUMAN

NCBI Description

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 Description

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

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; cell adhesion; liver development; 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