ADAMTS-13 is an enzyme responsible for the cleavage of von Willebrand factor under blood flow conditions.
A functional defect in the activity of this enzyme leads to the presence of high molecular weight isoforms of vWF, and thus leads to increased platelet aggregation mainly in areas of microvascularization.
This is the known major cause of Thrombotic Thrombocytopenic Purpura (TTP).
Principle of the test: the patient's plasma is incubated with a fragment of vWF.
The specific cleavage of this fragment by ADAMTS-13 is then detected with a labeled antibody directed against the cleaved fragment of VWF.
The intensity of the stain is directly proportional to the amount of cleaved substrate, and therefore to the activity of ADAMTS-13 in the plasma sample.
Each ADAMTS-13 deficiency should be confirmed with a quantitative assay system.
- 10 unit tests
- 10 pre-incubation tubes containing VWF fragments
- 1 vial x reagent R1 (4mL)
- 1 vial x reagent R2 (1mL)
- 1 vial x reagent R3 (1mL)
- 11 large pipettes (300 µL)
- 22 small pipettes (60 µL)
- 1 short Instruction pad
- 1 reference color chart