Mouse anti-Human. von Willebrand Factor Monoclonal Antibody | anti-VWF antibody

von Willebrand Factor/ Factor VIII Related-Ag

Cat. Num. AAA438215

• Gene Names: VWF; VWD; F8VWF
• Reactivity: Human.
• Applications: ELISA, Flow Cytometry, Functional Assay, Immunofluorescence, Western Blot, Immunoprecipitation, Immunohistochemistry
• 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); anti-VWF antibody

• Host: Mouse
• Reactivity: Human.
• Clonality: Monoclonal
• Isotype: IgG1, kappa
• Clone Number: VWF635
• Specificity: von Willebrand Factor (vWF) is a multimeric glycoprotein that is found in endothelial cells, plasma and platelets. It acts as a carrier protein for Factor VIII and promotes platelet adhesion and aggregation. vWF undergoes a variety of posttranslational modifications that influence the affinity and availability for Factor VIII, including cleavage of the propeptide and formation of N-terminal disulfide bonds. This antibody helps to establish the endothelial nature of some lesions of disputed histogenesis, e.g. Kaposi's sarcoma and cardiac myxoma. It is widely used for differentiating vascular lesions from those of other tissue differentiation within a panel of other vascular markers although not all tumors of endothelial differentiation contain this antigen.
• Form/Format: 200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml.
• Sequence Length: 2813

• Applicable Applications for anti-VWF antibody: ELISA (EIA), Flow Cytometry (FC/FACS), Immunofluorescence (IF), Western Blot (WB), Immunoprecipitation (IP), Immunohistology (IH)
• Application Notes: ELISA (For coating, order Ab without BSA); Flow Cytometry (0.5-1ug/million cells); Immunofluorescence (0.5-1ug/ml); Western Blotting (0.5-1.0ug/ml); Immunoprecipitation (0.5-1ug/500ug protein lysate); Immunohistology (Frozen & Formalin-fixed) (0.5-1.0ug/ml for 30 minutes at RT) (Staining of formalin-fixed tissues requires boiling tissue sections in 10mM Citrate Buffer, pH 6.0, for 10-20 min followed by cooling at RT for 20 minutes) Optimal dilution for a specific application should be determined.
• Localization: Cytoplasmic
• Positive Control: HUVEC cells or Tonsil
• Human Chromosomal Location: 12p13.31
• Immunogen: Recombinant human vWF fragment
• Quantity and Buffer: 0.5 ml at 200ug/ml with BSA and azide
• Preparation and Storage: Store at 2 to 8 degree C. Antibody is stable for 24 months.

Testing Data

(Formalin-fixed, paraffin-embedded human tonsil stained with vWF MAb (VWF635))

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; Q8TCE8; Q99806
• Molecular Weight: 250kDa
• 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; Secreted, signal peptide; Extracellular matrix; Cell adhesion; Motility/polarity/chemotaxis

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; blood coagulation; liver development; blood coagulation, intrinsic pathway; protein homooligomerization; cell-substrate adhesion; placenta development

Disease: Von Willebrand Disease, Type 3; Von Willebrand Disease, Type 1; Von Willebrand Disease, Type 2