ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif 13) is an enzyme (VWFcleaving protease or VWF-CP) that specifically cleaves von Willebrand factor (VWF), which induce platelet thrombus formation under high shear stress.
If the activity of ADAMTS13 is lowered for some reason, however, unusually large VWF multimers may accumulate, causing thrombosis due to platelet aggregation, which in turn may lead to TMA (thrombotic microangiopathy) such as TTP (thrombotic thrombocytopenic purpura).
- Stability 6 months after reconstitution (-20 ° C)
- 2 controls of 0.20 to 0.70 IU / mL. (depending on the lots).
Since October 2015, the controls and calibrators in the kit have been calibrated against the 1st WHO international standard (NIBSC code 12/252) in IU / mL.
- 2 vials x 0.5 mL lyophilized plasma