{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T01:26:31Z","timestamp":1782782791478,"version":"3.54.5"},"reference-count":125,"publisher":"Georg Thieme Verlag KG","issue":"03","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Thromb Hemost"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>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\u2013Schulman 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.<\/jats:p>","DOI":"10.1055\/s-0042-1758059","type":"journal-article","created":{"date-parts":[[2022,11,12]],"date-time":"2022-11-12T00:55:33Z","timestamp":1668214533000},"page":"284-294","source":"Crossref","is-referenced-by-count":21,"title":["From the Discovery of ADAMTS13 to Current Understanding of Its Role in Health and Disease"],"prefix":"10.1055","volume":"49","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4077-1796","authenticated-orcid":false,"given":"Adriana In\u00e9s","family":"Woods","sequence":"additional","affiliation":[{"name":"Laboratorio de Hemostasia y Trombosis, IMEX-CONICET-Academia Nacional de Medicina de Buenos Aires, CABA, Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2083-1269","authenticated-orcid":false,"given":"Juvenal","family":"Paiva","sequence":"additional","affiliation":[{"name":"Departamento de Hemostasia y Trombosis, Instituto de Investigaciones Hematol\u00f3gicas, Academia Nacional de Medicina de Buenos Aires, CABA, Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0431-1004","authenticated-orcid":false,"given":"Celia","family":"Dos Santos","sequence":"additional","affiliation":[{"name":"Laboratorio de Hemostasia y Trombosis, IMEX-CONICET-Academia Nacional de Medicina de Buenos Aires, CABA, Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7048-2031","authenticated-orcid":false,"given":"Mar\u00eda Fabiana","family":"Alberto","sequence":"additional","affiliation":[{"name":"Departamento de Hemostasia y Trombosis, Instituto de Investigaciones Hematol\u00f3gicas, Academia Nacional de Medicina de Buenos Aires, CABA, Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2971-9217","authenticated-orcid":false,"given":"Anal\u00eda","family":"S\u00e1nchez-Luceros","sequence":"additional","affiliation":[{"name":"Laboratorio de Hemostasia y Trombosis, IMEX-CONICET-Academia Nacional de Medicina de Buenos Aires, CABA, Argentina"},{"name":"Departamento de Hemostasia y Trombosis, Instituto de Investigaciones Hematol\u00f3gicas, Academia Nacional de Medicina de Buenos Aires, CABA, Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2022,11,11]]},"reference":[{"issue":"06","key":"ref1","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.1016\/j.hoc.2017.08.010","article-title":"Thrombotic microangiopathies (TTP, HUS, HELLP)","volume":"31","author":"S Kappler","year":"2017","journal-title":"Hematol Oncol Clin North Am"},{"issue":"02","key":"ref2","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1002\/jha2.154","article-title":"Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina","volume":"2","author":"C Dos Santos","year":"2021","journal-title":"eJHaem"},{"key":"ref3","first-page":"21","article-title":"Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease","volume":"24","author":"E Moschcowitz","year":"1924","journal-title":"Proc NY Pathol Soc"},{"issue":"06","key":"ref4","first-page":"542","article-title":"Thrombotic thrombocytopenic purpura; hemorrhagic diathesis with generalized platelet thromboses","volume":"2","author":"K Singer","year":"1947","journal-title":"Blood"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1182\/blood.V16.1.943.943","article-title":"Studies on thrombopoiesis. 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