ELISA von-Willebrand-Factor-Propeptide

Quicker 'ready-to-use-assay'; complete kit

Von Willebrand factor (vWF) is synthesized in endothelial cells and megakaryocytes as pre-pro-vWF. After various posttranslational modifications and cleavage of the signal peptide, the propeptide (vWF-PP) is also cleaved off by the protease furin in the trans-Golgi-system. A non-covalent complex of vWF and vWF-PP remains stored in Weibel-Palade bodies (endothelium) or in α-granules (megakaryocytes). Activation or stimulation of these cells will release the complex. VWF and vWF-PP are then metabolized differently. While vWF has a half-life of approx. 12 hours, vWF-PP is metabolized with a half-life of only approx. 2 hours.

The molar ratio of vWF-PP to vWF can be used as an indicator for the degradation of vWF. An increased ratio of vWF-PP / vWF indicates increased clearance of vWF. These are found in various patients with congenital vWF deficiency, but also in acquired vWF syndrome. An accurate knowledge of the clearance of vWF may influence the choice of therapy, in particular the need to administer vWF concentrates. Increased levels of vWF-PP or an abnormal ratio between vWF-PP and vWF may also be caused by activation of the endothelium or platelets.

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Literature

1. Haberichter SL. von Willebrand factor propeptide: biology and clinical utility. Blood 2015; 26:1753-61.
2. Sanders YV, Groeneveld D, et al. Von Willebrand Factor Propeptide and the phenotype classification of von Willebernd disease. Blood: 125:3008-19
3. O’Sullivan JM, Ward S, et al. von Willebrand factor clearance- biological mechanismas and clinical significance. BJ Haematol 2018;183, 185-195
4. Eikenboom JC, Tjenerberg P et al. Acquired von Willebrand syndrome: diagnostic problems and therapeutic options. Am J Hematol. 2007;82:55-8.
5. Habe K, Wada H et al. Plasma ADAMTS13, von Willebrand Factor (VWF), and VWF Propeptide Profiles in Patients With Connective Tissue Diseases and Antiphospholipid Syndrome. Clin Appl Thromb Hemost. 2017;23:622-630
6. Stufano F, Boscarino M, et al. Evaluation of the Utility of von Willebrand Factor Propeptide in the Differential Diagnosis of von Willebrand Disease and Acquired von Willebrand Syndrome. Semin Thromb Hemost. 2018 Jun 18.