This protease has the role of regulating the size of abnormally large multimers of von Willebrand factor.
The presence of anti-ADAMTS-13 autoantibodies inhibits the cleavage activity of this enzyme, causing accumulation of high molecular weight VWF multimer in plasma.
These autoantibodies are considered to be the main cause of PTT (Thrombotic Thrombocytopenic Purpura).
- Stability 6 months after reconstitution (-20 ° C)
- 1 control around 10 to 40 IU / mL (depending on the lots).
- 1 control around 60 to 100 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
