Human vWF-A2 Antibody
Human vWF-A2 Antibody Summary
Accession # P04275
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Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.
Preparation and Storage
- 12 months from date of receipt, -20 to -70 °C as supplied.
- 1 month, 2 to 8 °C under sterile conditions after reconstitution.
- 6 months, -20 to -70 °C under sterile conditions after reconstitution.
von Willebrand Factor (vWF) is a large, multimeric glycoprotein made by endothelial cells and megakaryocytes. The pre-pro-vWF protein contains 2813 amino acids (aa), which consists of 22 aa signal peptide, 741 aa propeptide, and mature vWF monomer of 2050 aa (1-4). The pro-vWF undergoes dimerization in the endoplasmic reticulum (ER) through C-terminal “cysteine-knot” (CK) domain. The pro-vWF dimmers are transported to Golgi and form multimers by forming disulfide bond in amino-terminal region of the mature form. The proteolytic processing of pro-region also occurs in Golgi. The matured vWF is stored in Weibel-Pallade bodies in endothelial cells and granules in megakaryocytes and platelets. The unusually-large vWF (ulvWF) multimers released from cells are very efficient in binding to platelets to form thrombus. The population of these highly active ulvWF multimers is controlled by a specific protease, ADAMTS13, which cleaves between residues Tyr1605 and Met1606 in the A2 domain of vWF. In the plasma, vWF appears as a series of large and intermediate multimers with molecular masses from several thousand to 500 kDa. vWF also performs hemostatic functions (3-5). In a high shear-stressed environment, vWF undergoes conformational change to expose a binding site for glycoprotein Ib alpha. As a result, vWF facilitates aggregation of platelets. In addition to platelet binding, vWF binds coagulation factor VIII to increase the lifetime of FVIII in plasma. The purified rhvWF-A2 contains the A2 domain of vWF.
- Sadler, J.E. (1998) Annu. Rev. Biochem. 67:395.
- Ruggeri, Z.M. (2003) Cur. Opin. Hemat. 10:142.
- Michiels, J.J. et al. (2006) Clin. Appl. Thromb. Hemost. 12:397.
- Groot, E. et al. (2007) Cur. Opin. Hemat. 14:284.
- Lenting, P.J. et al. (2007) J. Thromb. Haemos. 5:1353.
Citations for Human vWF-A2 Antibody
R&D Systems personnel manually curate a database that contains references using R&D Systems products. The data collected includes not only links to publications in PubMed, but also provides information about sample types, species, and experimental conditions.
Citations: Showing 1 - 2
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Down syndrome iPSC model: endothelial perspective on tumor development
Authors: M Perepitchk, Y Galat, IP Beletsky, PM Iannaccone, V Galat
Sample Types: Whole Cells
Autoregulation of von Willebrand factor function by a disulfide bond switch
Authors: D Butera, F Passam, L Ju, KM Cook, H Woon, C Aponte-San, E Gardiner, AK Davis, DA Murphy, A Bronowska, BM Luken, C Baldauf, S Jackson, R Andrews, F Gräter, PJ Hogg
Sci Adv, 2018;4(2):eaaq1477.
Sample Types: Protein
Applications: Binding Assay
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