- •VWF string detection with GPIb-beads instead of platelets.
- •Assay suitable for fluorescent applications.
- •Semi-automated, quantitative assessment of shear-dependent ADAMTS13 function.
- •Kinetic assays possible for recombinant and plasma ADAMTS13 under flow conditions.
- •Assay could foster and complement VWD and TTP research and diagnostics.
Material and Methods
Abbreviations:VWF (von Willebrand factor), WPB (Weibel-Palade Body), ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), GPIb (glycoprotein Ib), USS (Upshaw-Schulman Syndrome), TTP (Thrombotic Thrombocytopenic Purpura), RT (room temperature), PFA (paraformaldehyde), ROI (region of interest)
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- von Willebrand factor: the complex molecular genetics of a multidomain and multifunctional protein.J Thromb Haemost. 2011; 9: 209-215
- A novel calcium-binding site of von Willebrand factor A2 domain regulates its cleavage by ADAMTS13.Blood. 2011; 117: 4623-4631
- Cell biology of von Willebrand factor.Annu Rev Cell Biol. 1990; 6: 217-246
- Structures of glycoprotein Ibalpha and its complex with von Willebrand factor A1 domain.Science. 2002; 297: 1176-1179
- Local elongation of endothelial cell-anchored von Willebrand factor strings precedes ADAMTS13 protein-mediated proteolysis.J Biol Chem. 2011; 286: 36361-36367
- Microfluidic reveals generation of platelet-strings on tumor-activated endothelium.Thromb Haemost. 2007; 98: 283-286
- Extensive contacts between ADAMTS13 exosites and von Willebrand factor domain A2 contribute to substrate specificity.Blood. 2008; 112: 1713-1719
- Unraveling the scissile bond: how ADAMTS13 recognizes and cleaves von Willebrand factor.Blood. 2011; 118: 3212-3221
- The pathophysiology of von Willebrand disease: therapeutic implications.Thromb Res. 2011; 128: S3-S7
- Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura.Nature. 2001; 413: 488-494
- Thrombotic microangiopathies: towards a pathophysiology-based classification.Cardiovasc Hematol Disord Drug Targets. 2009; 9: 36-50
- von Willebrand disease type 2A phenotypes IIC, IID and IIE: A day in the life of shear-stressed mutant von Willebrand factor.Thromb Haemost. 2014; : 112
- Impact of mutations in the von Willebrand factor A2 domain on ADAMTS13-dependent proteolysis.Blood. 2006; 107: 2339-2345
- Human Experimentation. Code of Ethics of the World Medical Association. Declaration of Helsinki.Br Med J. 1964; 2: 177
- NIH Image to ImageJ: 25 years of image analysis.Nat Methods. 2012; 9: 671-675
- Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura.Blood. 2002; 100: 710-713
- ADAMTS-13 assays in thrombotic thrombocytopenic purpura.J Thromb Haemost. 2010; 8: 631-640
- Relation between ADAMTS13 activity and ADAMTS13 antigen levels in healthy donors and patients with thrombotic microangiopathies (TMA).Thromb Haemost. 2006; 95: 212-220