{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T18:04:42Z","timestamp":1792087482541,"version":"4.4.0"},"reference-count":27,"publisher":"Elsevier BV","issue":"8","license":[{"start":{"date-parts":[[2021,8,1]],"date-time":"2021-08-01T00:00:00Z","timestamp":1627776000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2021,8,1]],"date-time":"2021-08-01T00:00:00Z","timestamp":1627776000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2022,4,1]],"date-time":"2022-04-01T00:00:00Z","timestamp":1648771200000},"content-version":"vor","delay-in-days":243,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["jthjournal.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Thrombosis and Haemostasis"],"published-print":{"date-parts":[[2021,8]]},"DOI":"10.1111\/jth.15409","type":"journal-article","created":{"date-parts":[[2021,6,2]],"date-time":"2021-06-02T00:42:32Z","timestamp":1622594552000},"page":"1914-1921","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":76,"title":["ADAMTS13 regulation of VWF multimer distribution in severe COVID\u201019"],"prefix":"10.1016","volume":"19","author":[{"given":"Soracha E.","family":"Ward","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen","family":"Fogarty","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellie","family":"Karampini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Lavin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonja","family":"Schneppenheim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rita","family":"Dittmer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1259-0805","authenticated-orcid":false,"given":"Hannah","family":"Morrin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siobhan","family":"Glavey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cliona","family":"Ni Cheallaigh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colm","family":"Bergin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ignacio","family":"Martin\u2010Loeches","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick W.","family":"Mallon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerard F.","family":"Curley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ross I.","family":"Baker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich","family":"Budde","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1162-8739","authenticated-orcid":false,"given":"Jamie M.","family":"O\u2019Sullivan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0309-3313","authenticated-orcid":false,"given":"James S.","family":"O\u2019Donnell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niamh","family":"O\u2019Connell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary","family":"Byrne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liam","family":"Townsend","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalie L.","family":"McEvoy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Clarke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Boylan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Razi","family":"Alalqam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy P.","family":"Worrall","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Kelly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eoghan","family":"de Barra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger","family":"Preston","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dermot","family":"Kenny","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"4","key":"10.1111\/jth.15409_bb0010","doi-asserted-by":"crossref","first-page":"268","DOI":"10.7326\/M20-2003","article-title":"Autopsy findings and venous thromboembolism in patients with COVID\u201019: a prospective cohort study","volume":"173","author":"Wichmann","year":"2020","journal-title":"Ann Intern Med"},{"issue":"2","key":"10.1111\/jth.15409_bb0015","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1056\/NEJMoa2015432","article-title":"Pulmonary vascular endothelialitis, thrombosis, and angiogenesis in covid\u201019","volume":"383","author":"Ackermann","year":"2020","journal-title":"N Engl J Med"},{"issue":"10234","key":"10.1111\/jth.15409_bb0020","doi-asserted-by":"crossref","first-page":"1417","DOI":"10.1016\/S0140-6736(20)30937-5","article-title":"Endothelial cell infection and endotheliitis in COVID\u201019","volume":"395","author":"Varga","year":"2020","journal-title":"Lancet"},{"issue":"8","key":"10.1111\/jth.15409_bb0025","doi-asserted-by":"crossref","first-page":"e553","DOI":"10.1016\/S2352-3026(20)30215-5","article-title":"Endothelial cells orchestrate COVID\u201019 coagulopathy","volume":"7","author":"O\u2019Sullivan","year":"2020","journal-title":"Lancet Haematol"},{"key":"10.1111\/jth.15409_bb0030","doi-asserted-by":"crossref","first-page":"714","DOI":"10.1111\/bjh.17273","article-title":"Von Willebrand factor propeptide in severe coronavirus disease 2019 (COVID\u201019): evidence of acute and sustained endothelial cell activation","volume":"192","author":"Ward","year":"2020","journal-title":"Br J Haematol"},{"issue":"8","key":"10.1111\/jth.15409_bb0035","doi-asserted-by":"crossref","first-page":"e575","DOI":"10.1016\/S2352-3026(20)30216-7","article-title":"Endotheliopathy in COVID\u201019\u2010associated coagulopathy: evidence from a single\u2010centre, cross\u2010sectional study","volume":"7","author":"Goshua","year":"2020","journal-title":"Lancet Haematol"},{"issue":"9","key":"10.1111\/jth.15409_bb0040","doi-asserted-by":"crossref","first-page":"1192","DOI":"10.1182\/blood-2015-07-654921","article-title":"A novel role for von Willebrand factor in the pathogenesis of experimental cerebral malaria","volume":"127","author":"O\u2019Regan","year":"2016","journal-title":"Blood"},{"issue":"2","key":"10.1111\/jth.15409_bb0045","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1111\/jth.15191","article-title":"The ADAMTS13\u2010von Willebrand factor axis in COVID\u201019 patients","volume":"19","author":"Mancini","year":"2021","journal-title":"J Thromb Haemost"},{"key":"10.1111\/jth.15409_bb0050","first-page":"3","article-title":"Circulating Von Willebrand factor and high molecular weight multimers as markers of endothelial injury predict COVID\u201019 in\u2010hospital mortality","volume":"1","author":"Philippe","year":"2021","journal-title":"Angiogenesis"},{"key":"10.1111\/jth.15409_bb0055","doi-asserted-by":"crossref","first-page":"e540","DOI":"10.3324\/haematol.2020.262345","article-title":"Dramatic presentation of acquired thrombotic thrombocytopenic purpura associated with COVID\u201019","volume":"105","author":"Capecchi","year":"2020","journal-title":"Haematologica"},{"issue":"6","key":"10.1111\/jth.15409_bb0060","doi-asserted-by":"crossref","first-page":"1089","DOI":"10.1007\/s00134-020-06062-x","article-title":"High risk of thrombosis in patients with severe SARS\u2010CoV\u20102 infection: a multicenter prospective cohort study","volume":"46","author":"Helms","year":"2020","journal-title":"Intensive Care Med"},{"issue":"7","key":"10.1111\/jth.15409_bb0065","doi-asserted-by":"crossref","first-page":"1738","DOI":"10.1111\/jth.14850","article-title":"Hypercoagulability of COVID\u201019 patients in intensive care unit: a report of thromboelastography findings and other parameters of hemostasis","volume":"18","author":"Panigada","year":"2020","journal-title":"J Thromb Haemost"},{"issue":"14","key":"10.1111\/jth.15409_bb0070","doi-asserted-by":"crossref","first-page":"1585","DOI":"10.1182\/blood-2018-09-874636","article-title":"Increased galactose expression and enhanced clearance in patients with low von Willebrand factor","volume":"133","author":"Aguila","year":"2019","journal-title":"Blood"},{"issue":"5","key":"10.1111\/jth.15409_bb0075","doi-asserted-by":"crossref","first-page":"1472","DOI":"10.3324\/haematol.2020.262634","article-title":"Hemostatic alterations in COVID\u201019","volume":"106","author":"Peyvandi","year":"2021","journal-title":"Haematologica"},{"key":"10.1111\/jth.15409_bb0080","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1016\/j.thromres.2021.02.012","article-title":"Recombinant ADAMTS13 reduces abnormally up\u2010regulated von Willebrand factor in plasma from patients with severe COVID\u201019","volume":"201","author":"Turecek","year":"2021","journal-title":"Thromb Res"},{"issue":"5","key":"10.1111\/jth.15409_bb0085","doi-asserted-by":"crossref","first-page":"e512","DOI":"10.1097\/CCM.0000000000004918","article-title":"von Willebrand factor multimer formation contributes to immunothrombosis in coronavirus disease 2019","volume":"49","author":"Doevelaar","year":"2021","journal-title":"Crit Care Med"},{"issue":"2","key":"10.1111\/jth.15409_bb0090","first-page":"105","article-title":"Thrombotic thrombocytopenic purpura: understanding a disease no longer rare","volume":"316","author":"Moake","year":"1998","journal-title":"Am J Med Sci"},{"issue":"8","key":"10.1111\/jth.15409_bb0095","doi-asserted-by":"crossref","first-page":"1460","DOI":"10.1111\/j.1538-7836.2004.00830.x","article-title":"Variations among normal individuals in the cleavage of endothelial\u2010derived ultra\u2010large von Willebrand factor under flow","volume":"2","author":"Dong","year":"2004","journal-title":"J Thromb Haemost"},{"issue":"13","key":"10.1111\/jth.15409_bb0100","doi-asserted-by":"crossref","first-page":"2666","DOI":"10.1182\/blood-2009-09-241547","article-title":"Expression of terminal alpha2\u20106\u2010linked sialic acid on von Willebrand factor specifically enhances proteolysis by ADAMTS13","volume":"115","author":"McGrath","year":"2010","journal-title":"Blood"},{"issue":"7","key":"10.1111\/jth.15409_bb0105","doi-asserted-by":"crossref","first-page":"1018","DOI":"10.1111\/jth.14471","article-title":"von Willebrand factor sialylation\u2014A critical regulator of biological function","volume":"17","author":"Ward","year":"2019","journal-title":"J Thromb Haemost"},{"issue":"5","key":"10.1111\/jth.15409_bb0110","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1161\/ATVBAHA.116.308524","article-title":"Plasmin cleaves von willebrand factor at K1491\u2013R1492 in the A1\u2013A2 linker region in a shear\u2010 and glycan\u2010dependent manner in vitro","volume":"37","author":"Brophy","year":"2017","journal-title":"Arterioscler Thromb Vasc Biol"},{"key":"10.1111\/jth.15409_bb0115","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1161\/ATVBAHA.120.315338","article-title":"COVID\u201019 and sepsis are associated with different abnormalities in plasma procoagulant and fibrinolytic activity","volume":"41","author":"Bouck","year":"2020","journal-title":"Arterioscler Thromb Vasc Biol"},{"issue":"25","key":"10.1111\/jth.15409_bb0120","doi-asserted-by":"crossref","first-page":"4107","DOI":"10.1182\/blood-2013-04-496851","article-title":"Altered glycosylation of platelet\u2010derived von Willebrand factor confers resistance to ADAMTS13 proteolysis","volume":"122","author":"McGrath","year":"2013","journal-title":"Blood"},{"issue":"1","key":"10.1111\/jth.15409_bb0125","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1182\/blood-2004-01-0107","article-title":"Effects of inflammatory cytokines on the release and cleavage of the endothelial cell\u2010derived ultralarge von Willebrand factor multimers under flow","volume":"104","author":"Bernardo","year":"2004","journal-title":"Blood"},{"issue":"3","key":"10.1111\/jth.15409_bb0130","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1182\/blood-2004-12-4856","article-title":"Thrombospondin\u20101 controls vascular platelet recruitment and thrombus adherence in mice by protecting endothelial VWF from cleavage by ADAMTS13","volume":"107","author":"Bonnefoy","year":"2006","journal-title":"Blood"},{"issue":"4","key":"10.1111\/jth.15409_bb0135","doi-asserted-by":"crossref","first-page":"594","DOI":"10.1111\/bjh.16553","article-title":"Platelet factor 4 inhibits ADAMTS13 activity and regulates the multimeric distribution of von Willebrand factor","volume":"190","author":"Nazy","year":"2020","journal-title":"Br J Haematol"},{"issue":"3","key":"10.1111\/jth.15409_bb0140","doi-asserted-by":"crossref","first-page":"1085","DOI":"10.1182\/blood-2004-03-1101","article-title":"Proteolytic inactivation of ADAMTS13 by thrombin and plasmin","volume":"105","author":"Crawley","year":"2005","journal-title":"Blood"}],"container-title":["Journal of Thrombosis and Haemostasis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jth.15409","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/jth.15409","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1538783622018657?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1538783622018657?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jth.15409","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T03:32:32Z","timestamp":1761535952000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1538783622018657"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,8]]},"references-count":27,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2021,8]]}},"alternative-id":["S1538783622018657"],"URL":"https:\/\/doi.org\/10.1111\/jth.15409","relation":{},"ISSN":["1538-7836"],"issn-type":[{"value":"1538-7836","type":"print"}],"subject":[],"published":{"date-parts":[[2021,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"ADAMTS13 regulation of VWF multimer distribution in severe COVID\u201019","name":"articletitle","label":"Article Title"},{"value":"Journal of Thrombosis and Haemostasis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1111\/jth.15409","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2021 The Authors. Journal of Thrombosis and Haemostasis published by ELSEVIER INC. on behalf of International Society on Thrombosis and Haemostasis","name":"copyright","label":"Copyright"}]}}