Technofluor® ADAMTS-13 Activity
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The Technofluor® ADAMTS13 Activity test kit is based on a fluorogenic von Willebrand Factor (vWF) substrate for the quantitative determination of ADAMTS-13 activity in citrated human plasma in a fully automated method.
vWF promotes clot formation by tethering platelets to the site of blood vessel injury and may contribute to platelet aggregation. It is comprised of subunits of various sizes, termed multimers, whose adhesiveness increases with their size. The enzyme ADAMTS13 (A Disintegrin-like And Metalloproteinase with ThromboSpondin type 1 motif 13) acts as a gatekeeper against the generation of highly thrombotic ultra-large vWF multimers by regulating their size via cleavage at a specific site, thereby breaking down large multimers into smaller, less thrombogenic forms. Some ultra-large multimers can become millimeters in length if unregulated. In ADAMTS13 deficient states, unsually large hyperfunctional vWF multimers can accumulate, leading to microvascular thrombosis and organ damage or failure due to vWF-platelet aggregates. This may lead to a microangipathic hemolytic anemia as red blood cells are sheared as they travel past and through the microthrombi. This deficiency can be either acquired or hereditary, the former commonly due to the presence of ADAMTS13 autoantibodies, and is termed thrombotic thrombocytopenic purpura (TTP). To learn more, read here.
In the Technofluor ADAMTS13 assay, ADAMTS-13 from the plasma sample cleaves a VWF73 based substrate, releasing a dark quencher. With the quencher no longer present, the energy transfer from an adjacent emitter is allowed to stimulate the fluorophore allowing emission of fluorescence. The fluorescent signal generated is proportional to the amount of ADAMTS-13 activity is present in the sample.
| Reagent | Description | |
|---|---|---|
| 2x 1 mL | Substrate (SUB) | ADAMTS-13 specific substrate with dark quencher |
| 2x 3 mL | Buffer (BUF) | ADAMTS-13 buffer for sample dilution |
Stability and Storage
| Reagent/Content | Ceveron s100 (open vial) | 2-8 Celsius (closed vial) | < -20 Celsius (closed vial) |
|---|---|---|---|
| Substrate (SUB) | 3 Days | 1 Month | 2 Months |
| Buffer (BUF) | 3 Days | 2 Months | Do Not Freeze |
Normal Ranges
| Reference Range: | 0.6 – 1.21 IU/mL (Equivalent to 60 – 121%) |
|---|---|
| Lower Limit of Detection: | 0.004 IU/mL |
| Linear Range: | 0.01 – 1.25 IU/mL |
Precision and Reproducibility
| Sample | Value (%) | %CV within run | %CV between run |
|---|---|---|---|
| Plasma Sample 1 | 0.075 | 10.60% | 11.60% |
| Plasma Sample 2 | 0.154 | 11.70% | 11.70% |
| Plasma Sample 3 | 0.407 | 5.60% | 6.00% |
| Plasma Sample 4 | 0.775 | 5.60% | 6.40% |
| Plasma Sample 5 | 1.203 | 3.30% | 5.30% |