Mouse anti-Human von Willebrand Factor/Factor VIII Related-Ag Antibody | anti-VWF antibody

von Willebrand Factor/Factor VIII Related-Ag (Endothelial Marker)

Cat. Num. AAA4381341

• Gene Names: VWF; VWD; F8VWF
• Reactivity: Human
• Applications: Immunohistochemistry
• Purity: Purified Ab with BSA and Azide at 200ug/ml OR Purified Ab WITHOUT BSA and Azide at 1.0mg/ml
• Synonyms: von Willebrand Factor/Factor VIII Related-Ag; von Willebrand Factor/Factor VIII Related-Ag (Endothelial Marker); Coagulation Factor VIII; Factor VIII Related Antigen; F8VWF; von Willebrand Antigen 2; von Willebrand Disease (vWD); anti-VWF antibody

• Host: Mouse
• Reactivity: Human
• Isotype: IgG1, kappa
• Clone Number: rVWF/2480
• 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.
• Purity/Purification: Purified Ab with BSA and Azide at 200ug/ml OR Purified Ab WITHOUT BSA and Azide at 1.0mg/ml
• 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: Immunohistochemistry (IHC) (Formalin-fixed)
• Application Notes: Immunohistochemistry (Formalin-fixed) (1-2ug/ml for 30 minutes at RT) (Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95 °C followed by cooling at RT for 20 minutes); Optimal dilution for a specific application should be determined.
• Positive Control: HUVEC cells. Tonsil (IHC).
• Immunogen: Recombinant full-length human VWF protein
• Cellular Localization: Cytoplasmic
• Antibody Type: Recombinant Mouse Monoclonal Antibody
• Preparation and Storage: Antibody without azide - store at -20 to -80 °C.

Immunohistochemistry (IHC)

(Formalin-fixed, paraffin-embedded human Tonsil stained with vWF Recombinant Mouse Monoclonal Antibody (rVWF/2480).)

SDS-Page

(SDS-PAGE Analysis Purified vWF Recombinant Mouse Monoclonal Antibody (rVWF/2480). Confirmation of Purity and Integrity of Antibody.)

References

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

NCBI and Uniprot Product Information

• NCBI GI #: 89191868
• NCBI GeneID: 7450
• NCBI Accession #: NP_000543.2
• NCBI GenBank Nucleotide #: NP_000543.2
• UniProt Accession #: P04275
• 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
• 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

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]

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