Goat von Willebrand Factor Polyclonal Antibody | anti-VWF antibody

Goat anti-human von Willebrand Factor, Affinity-Purified IgG

Cat. Num. AAA512175

• Gene Names: VWF; F8VWF
• Purity: Affinity purified IgG.; Vial containing ml of IgG purified by affinity chromatography on immobilized vWF. Total protein is 0.5 mg.
• Synonyms: von Willebrand Factor; Polyclonal Antibody; Goat anti-human von Willebrand Factor; Affinity-Purified IgG; human; anti-VWF antibody

• Host: Goat
• Clonality: Polyclonal
• Specificity: This antibody is specific for vWF as demonstrated by immunoelectrophoresis and ELISA.
• Purity/Purification: Affinity purified IgG.; Vial containing ml of IgG purified by affinity chromatography on immobilized vWF. Total protein is 0.5 mg.
• Form/Format: Affinity-purified IgG (APIgG), clear liquid.
• Concentration: APIgG concentration is mg/ml, determined by absorbance using an extinction coefficient (E1%280) of 13.4. (varies by lot)

• Application Notes: Suitable as a source of enriched antibodies to vWF.
• Immunogen: Human vWF purified from plasma.
• Buffer: 10 mM HEPES, pH 7.4, 150 mM NaCl, 50% (v/v) glycerol.
• Neutralizing Activity: Not determined
• Preparation and Storage: Store between -10 and -20 degree C. Product will become viscous but will not freeze. Avoid storage in frost-free freezers. Keep vial tightly capped. Allow product to warm to room temperature and gently mix before use.

Related Product Information for anti-VWF antibody

von Willebrand Factor (vWF, also previously referred to as Factor VIII related antigen) is a large adhesive protein produced in endothelial cells and megakaryocytes. There are two critical functions of vWF, the first being its involvement in the process of platelet adhesion and aggregation through interaction with platelet receptor glycoprotein Ib, the second being the binding and stabilization of Factor VIII (antihemophilic factor) for secretion and transport in plasma. The vWF precursor protein is synthesized with a 95,000 dalton propeptide (also known as vWF antigen-II), believed to be involved in the intracellular multimerization of the vWF subunits. The mature vWF multimers are then packed into storage organelles within the cell (Weibel-Palade bodies) after which the propeptide is cleaved and released. vWF circulates as multimers of disulphide linked 220,000 dalton subunits and the molecular weight of these multimers ranges from 0.5- 20 million daltons. The plasma concentration of vWF is typically 10 ug/ml, but increased levels are often observed in pregnancy and other conditions of physiological stress. von Willebrand's disease (vWD) is perhaps the most common inherited bleeding disorder in humans and is the result of either quantitative deficiencies of vWF (vWD Types I & III), or one of a number of qualitative disorders of vWF structure and function (vWD Type II).

References

1. Montgomery RR, Coller BS, in Hemostasis and Thrombosis, 3rd Edition, eds. RW Colman, J Hirsh, VJ Marder and EW Salzman, pp. 134- 168, J.B. Lippincott Co., Philadelphia PA, USA, 1994.
2. Sadler JE; von Willebrand Factor (Minireview); JBC 266:34, pp 22777- 22780, 1991.

NCBI and Uniprot Product Information

• NCBI GI #: 50950127
• NCBI GeneID: 399544
• NCBI Accession #: NP_001002932.1
• NCBI GenBank Nucleotide #: NM_001002932.1
• UniProt Accession #: Q28295; Q28311; Q9TSI4
• Molecular Weight: 309533 Da
• NCBI Official Full Name: von Willebrand factor
• NCBI Official Symbol: VWF
• NCBI Official Synonym Symbols: 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
• UniProt Entry Name: VWF_CANFA

Uniprot Description

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, glycoprotein Ibalpha/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.2

Subcellular location: Secreted. Secreted › extracellular space › extracellular matrix. Note: Localized to storage granules. Ref.2

Tissue specificity: Plasma.

Domain: The propeptide 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

By similarity.N- and O-glycosylated

Involvement in disease: Note=Defects in VWF are the cause of von Willebrand disease (VWD) in the Scottish Terrier. VWD is characterized by frequent bleeding. Type I VWD is associated with a deficiency of VWF; type II by normal to decreased plasma level of VWF; type III by a virtual absence of VWF. Ref.4

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.