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ADAMTS-13 assays in thrombotic thrombocytopenic purpura

Peyvandi, F; Palla, R; Lotta, LA; Mackie, I; Scully, MA; Machin, SJ; (2010) ADAMTS-13 assays in thrombotic thrombocytopenic purpura. J THROMB HAEMOST , 8 (4) 631 - 640. 10.1111/j.1538-7836.2010.03761.x.

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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.

Type: Article
Title: ADAMTS-13 assays in thrombotic thrombocytopenic purpura
DOI: 10.1111/j.1538-7836.2010.03761.x
Keywords: ADAMTS-13, assays, clinical utility, standardization, VON-WILLEBRAND-FACTOR, FACTOR-CLEAVING PROTEASE, HEMOLYTIC-UREMIC SYNDROME, ANTI-ADAMTS13 ANTIBODIES, FRETS-VWF73 ASSAY, ENZYME-IMMUNOASSAY, PROGNOSTIC VALUE, PLASMA-EXCHANGE, IGG ANTIBODIES, SUBSTRATE
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute > Research Department of Haematology
URI: http://discovery.ucl.ac.uk/id/eprint/1324269
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