ADAMTS 13 Specific Quenching Fluorescent Substrate


It has been shown that thrombotic thrombocytopenic purpura (TTP) is due to reduced function of ADAMTS-13, a VWF-cleaving enzyme in plasma. A marked reduction in ADAMTS-13 activity due to congenital or acquired factors results in accumulation of abnormally high molecular weight VWFs in blood, causing platelet hyperaggregation in small blood vessels.

Kokame et al. of the National Cerebral and Cardiovascular Center Research Institute revealed that the shortest region that serves as a specific substrate for ADAMTS-13 is 73 residues corresponding to Asp1596-Arg1668 of VWFs.1)
In addition, they successfully developed FRETS-VWF73 (shown below), a quenching fluorescent substrate with a fluorescent group (2-(N-methylamino) benzoyl, Nma) and a quenching group (2,4-dinitrophenyl, Dnp) around the cleavage site. As a result, ADAMTS-13 activity can be measured within 1 hour by detecting the fluorescence of FRETS-VWF73 in plasma over time.2)

TTP may be difficult to distinguish from hemolytic uremic syndrome (HUS) and disseminated intravascular coagulation (DIC), which manifest with similar symptoms. FRETS-VWF73 is expected to contribute to the rapid determination of ADAMTS-13 activity, a measure used in the definitive diagnosis of TTP. It may also be very useful for basic research on ADAMTS-13, which remains poorly understood.

For research use or further manufacturing use only. Not for use in diagnostic procedures.

Product content may differ from the actual image due to minor specification changes etc.

If the revision of product standards and packaging standards has been made, there is a case where the actual product specifications and images are different.


Hours of Operation: 8:00 - 17:00 (EST)For other hours than the above, please contact us via the inquiry form.