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

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.

Acute Hepatitis E Induced the First Episode of Immune-Mediated Thrombotic Thrombocytopenic Purpura: The First Case Report

“…Lipids might block the device membrane in a semiquantitative assay. 14 Our ADAMTS-13 activity tests used VWF:CB assays, and our patient did not have hyperlipidaemia. Despite the patient’s hyperbilirubinemia, the results of ADAMTS-13 activity are credible.…”

2024 Eberhard F. Mammen Award Announcements: Part I—Most Popular Articles

“…Consequently, the most popular papers from each category are identified separately. Tables 3 and 4 respectively list the top 25 downloaded articles from STH in 2023 (from 2022 and 2023 inclusive), as eligible for either the “free access” ( Table 3 ) 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 or “general category” ( Table 4 ) 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 awards.…”

“…The publisher of STH, Thieme, had in the past provided most material related to COVID-19 as free to download, and so COVID19-related material is less likely to be itemized in the “general category” list of most popular. 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 The 25 most popular non-free to download papers are listed in Table 4 . The first two papers listed are the official winners of the 2024 Most Popular in the general category.…”

Celebrating 50 Years of Seminars in Thrombosis and Hemostasis—Part II

“…A change in direction follows, with Woods and colleagues 17 taking us on a journey from the discovery of ADAMTS13 (a disintegrin-like metalloprotease domain with thrombospondin type 1 motifs, member 13) to current understanding of its role in health and disease. ADAMTS13 is a protease of crucial importance in the regulation of the size of von Willebrand factor (VWF) multimers.…”