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Semin Thromb Hemost 2023; 49(03): 284-294
DOI: 10.1055/s-0042-1758059
Review Article

From the Discovery of ADAMTS13 to Current Understanding of Its Role in Health and Disease

Adriana Inés Woods 
1   Laboratorio de Hemostasia y Trombosis, IMEX-CONICET-Academia Nacional de Medicina de Buenos Aires, CABA, Argentina
,
Juvenal Paiva 
2   Departamento de Hemostasia y Trombosis, Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina de Buenos Aires, CABA, Argentina
,
Celia Dos Santos 
1   Laboratorio de Hemostasia y Trombosis, IMEX-CONICET-Academia Nacional de Medicina de Buenos Aires, CABA, Argentina
,
María Fabiana Alberto 
2   Departamento de Hemostasia y Trombosis, Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina de Buenos Aires, CABA, Argentina
,
Analía Sánchez-Luceros 
1   Laboratorio de Hemostasia y Trombosis, IMEX-CONICET-Academia Nacional de Medicina de Buenos Aires, CABA, Argentina
2   Departamento de Hemostasia y Trombosis, Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina de Buenos Aires, CABA, Argentina
› Author Affiliations Funding Our work is supported by CONICET, Fundación Rene Barón, and Academia Nacional de Medicina (Buenos Aires), Argentina.
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Abstract

ADAMTS13 (a disintegrin-like metalloprotease domain with thrombospondin type 1 motif, member 13) is a protease of crucial importance in the regulation of the size of von Willebrand factor multimers. Very low ADAMTS13 activity levels result in thrombotic thrombocytopenic purpura, a rare and life-threatening disease. The mechanisms involved can either be acquired (immune-mediated thrombotic thrombocytopenic purpura [iTTP]) or congenital (cTTP, Upshaw–Schulman syndrome) caused by the autosomal recessive inheritance of disease-causing variants (DCVs) located along the ADAMTS13 gene, which is located in chromosome 9q34. Apart from its role in TTP, and as a regulator of microthrombosis, ADAMTS13 has begun to be identified as a prognostic and/or diagnostic marker of other diseases, such as those related to inflammatory processes, liver damage, metastasis of malignancies, sepsis, and different disorders related to angiogenesis. Since its first description almost 100 years ago, the improvement of laboratory tests and the description of novel DCVs along the ADAMTS13 gene have contributed to a better and faster diagnosis of patients under critical conditions. The ability of ADAMTS13 to dissolve platelet aggregates in vitro and its antithrombotic properties makes recombinant human ADAMTS13 treatment a potential therapeutic approach targeting not only patients with cTTP but also other medical conditions.

Keywords

ADAMTS13 - thrombotic thrombocytopenic purpura - Upshaw–Schulman syndrome - genotypic studies - phenotypic studies


Publication History

Article published online:
11 November 2022

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