{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T16:51:04Z","timestamp":1789750264412,"version":"4.0.1"},"reference-count":53,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2013,9,1]],"date-time":"2013-09-01T00:00:00Z","timestamp":1377993600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2013,9,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>This study addressed the contribution of ADAMTS13 deficiency to complement activation in thrombotic thrombocytopenic purpura (TTP). Renal tissue and blood samples were available from 12 TTP patients. C3 and C5b-9 deposition were demonstrated in the renal cortex of two TTP patients, by immunofluorescence and immunohistochemistry, respectively. C3 was also demonstrated in the glomeruli of Shiga toxin-2\u2013treated Adamts13\u2212\/\u2212 mice (n = 6 of 7), but less in mice that were not Shiga toxin-2 treated (n = 1 of 8, p &amp;lt; 0.05) or wild-type mice (n = 0 of 7). TTP patient plasma (n = 9) contained significantly higher levels of complement-coated endothelial microparticles than control plasma (n = 13), as detected by flow cytometry. Exposure of histamine-stimulated primary glomerular endothelial cells to platelet-rich plasma from patients, or patient platelet-poor plasma combined with normal platelets, in a perfusion system, under shear, induced C3 deposition on von Willebrand factor\u2013platelet strings (on both von Willebrand factor and platelets) and on endothelial cells. Complement activation occurred via the alternative pathway. No C3 was detected when cells were exposed to TTP plasma that was preincubated with EDTA or heat-inactivated, or to control plasma. In the perfusion system, patient plasma induced more release of C3- and C9-coated endothelial microparticles compared with control plasma. The results indicate that the microvascular process induced by ADAMTS13 deficiency triggers complement activation on platelets and the endothelium, which may contribute to formation of thrombotic microangiopathy.<\/jats:p>","DOI":"10.4049\/jimmunol.1301221","type":"journal-article","created":{"date-parts":[[2013,7,23]],"date-time":"2013-07-23T03:51:30Z","timestamp":1374551490000},"page":"2184-2193","source":"Crossref","is-referenced-by-count":57,"title":["Complement Activation Associated with ADAMTS13 Deficiency in Human and Murine Thrombotic Microangiopathy"],"prefix":"10.1093","volume":"191","author":[{"given":"Ramesh","family":"Tati","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Clinical Sciences Lund, Lund University , 22185 Lund,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ann-Charlotte","family":"Kristoffersson","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Clinical Sciences Lund, Lund University , 22185 Lund,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne-lie","family":"St\u00e5hl","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Clinical Sciences Lund, Lund University , 22185 Lund,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johan","family":"Rebetz","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Clinical Sciences Lund, Lund University , 22185 Lund,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Pediatrics, University of Iowa College of Medicine , Iowa City, IA 52242"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Licht","sequence":"additional","affiliation":[{"name":"Division of Nephrology, The Hospital for Sick Children and University of Toronto , Toronto, Ontario M5G 1X8,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Motto","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Pediatrics, University of Iowa College of Medicine , Iowa City, IA 52242"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Karpman","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Clinical Sciences Lund, Lund University , 22185 Lund,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2013,9,1]]},"reference":[{"key":"2025032405230820700_r1","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1046\/j.1523-1755.2001.060003831.x","article-title":"Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura","volume":"60","author":"Ruggenenti","year":"2001","journal-title":"Kidney Int."},{"key":"2025032405230820700_r2","doi-asserted-by":"crossref","first-page":"3097","DOI":"10.1182\/blood.V89.9.3097","article-title":"Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura","volume":"89","author":"Furlan","year":"1997","journal-title":"Blood"},{"key":"2025032405230820700_r3","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1038\/35097008","article-title":"Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura","volume":"413","author":"Levy","year":"2001","journal-title":"Nature"},{"key":"2025032405230820700_r4","doi-asserted-by":"crossref","first-page":"1585","DOI":"10.1056\/NEJM199811263392203","article-title":"Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura","volume":"339","author":"Tsai","year":"1998","journal-title":"N. 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