Abstract
ADAMTS-13, the thirteenth member of the ADAMTS (A Disintegrin And Metalloprotease with Thrombo-Spondin 1 repeats) family, is the plasma metalloprotease responsible for regulating the multimeric structure of VWF. In congenital or acquired deficiency it is actively involved in the pathophysiology of thrombotic thrombocytopenic purpura (TTP), a rare but life threatening disease characterized by microangiopathic haemolytic anaemia and consumptive thrombocytopenia leading to disseminated microvascular thrombosis and variable signs and symptoms of organ ischemia and damage. In the last few years, a number of in house and commercial laboratory assays for ADAMTS-13 and its autoantibodies have been developed. The features and clinical utility of ADAMTS-13 assays are summarized in this review.
| Original language | English |
|---|---|
| Pages (from-to) | 631-640 |
| Number of pages | 10 |
| Journal | Journal of Thrombosis and Haemostasis |
| Volume | 8 |
| Issue number | 4 |
| DOIs | |
| Publication status | Published - Apr 2010 |
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Keywords
- ADAMTS-13
- Assays
- Clinical utility
- Standardization
ASJC Scopus subject areas
- Hematology
Cite this
ADAMTS-13 assays in thrombotic thrombocytopenic purpura. / Peyvandi, F.; Palla, R.; Lotta, L. A.; Mackie, I.; Scully, M. A.; Machin, S. J.
In: Journal of Thrombosis and Haemostasis, Vol. 8, No. 4, 04.2010, p. 631-640.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - ADAMTS-13 assays in thrombotic thrombocytopenic purpura
AU - Peyvandi, F.
AU - Palla, R.
AU - Lotta, L. A.
AU - Mackie, I.
AU - Scully, M. A.
AU - Machin, S. J.
PY - 2010/4
Y1 - 2010/4
N2 - ADAMTS-13, the thirteenth member of the ADAMTS (A Disintegrin And Metalloprotease with Thrombo-Spondin 1 repeats) family, is the plasma metalloprotease responsible for regulating the multimeric structure of VWF. In congenital or acquired deficiency it is actively involved in the pathophysiology of thrombotic thrombocytopenic purpura (TTP), a rare but life threatening disease characterized by microangiopathic haemolytic anaemia and consumptive thrombocytopenia leading to disseminated microvascular thrombosis and variable signs and symptoms of organ ischemia and damage. In the last few years, a number of in house and commercial laboratory assays for ADAMTS-13 and its autoantibodies have been developed. The features and clinical utility of ADAMTS-13 assays are summarized in this review.
AB - ADAMTS-13, the thirteenth member of the ADAMTS (A Disintegrin And Metalloprotease with Thrombo-Spondin 1 repeats) family, is the plasma metalloprotease responsible for regulating the multimeric structure of VWF. In congenital or acquired deficiency it is actively involved in the pathophysiology of thrombotic thrombocytopenic purpura (TTP), a rare but life threatening disease characterized by microangiopathic haemolytic anaemia and consumptive thrombocytopenia leading to disseminated microvascular thrombosis and variable signs and symptoms of organ ischemia and damage. In the last few years, a number of in house and commercial laboratory assays for ADAMTS-13 and its autoantibodies have been developed. The features and clinical utility of ADAMTS-13 assays are summarized in this review.
KW - ADAMTS-13
KW - Assays
KW - Clinical utility
KW - Standardization
UR - http://www.scopus.com/inward/record.url?scp=77952734070&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77952734070&partnerID=8YFLogxK
U2 - 10.1111/j.1538-7836.2010.03761.x
DO - 10.1111/j.1538-7836.2010.03761.x
M3 - Article
C2 - 20088924
AN - SCOPUS:77952734070
VL - 8
SP - 631
EP - 640
JO - Journal of Thrombosis and Haemostasis
JF - Journal of Thrombosis and Haemostasis
SN - 1538-7933
IS - 4
ER -