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Soliris (eculizumab) [Package Insert]. US Food Drug Adm website https\/\/www.accessdata.fda.gov\/drugsatfda_docs\/label\/2019\/125166s431lbl.pdf. 2019; Revised June, 2019. Accessed September, 2019. LP-."},{"key":"pcbi.1008139.ref074","doi-asserted-by":"crossref","first-page":"207","DOI":"10.5045\/br.2017.52.3.207","article-title":"Efficacy of eculizumab in paroxysmal nocturnal hemoglobinuria patients with or without aplastic anemia: prospective study of a Korean PNH cohort","volume":"52","author":"CW Choi","year":"2017","journal-title":"Blood Res"},{"key":"pcbi.1008139.ref075","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1182\/blood-2015-03-637645","article-title":"Eculizumab treatment efficiently prevents C5 cleavage without C5a generation in vivo","volume":"126","author":"EB Volokhina","year":"2015","journal-title":"Blood"},{"key":"pcbi.1008139.ref076","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.molimm.2017.05.021","article-title":"Eculizumab-C5 complexes express a C5a neoepitope in vivo: Consequences for interpretation of patient complement analyses","volume":"89","author":"PH Nilsson","year":"2017","journal-title":"Mol Immunol"},{"key":"pcbi.1008139.ref077","doi-asserted-by":"crossref","first-page":"970","DOI":"10.1182\/blood-2016-08-732800","article-title":"Incomplete inhibition by eculizumab: mechanistic evidence for residual C5 activity during strong complement activation","volume":"129","author":"MJ Harder","year":"2017","journal-title":"Blood"},{"key":"pcbi.1008139.ref078","doi-asserted-by":"crossref","first-page":"635670","DOI":"10.1155\/2015\/635670","article-title":"Clinical Applications of Hemolytic Markers in the Differential Diagnosis and Management of Hemolytic Anemia","volume":"2015","author":"W Barcellini","year":"2015","journal-title":"Dis Markers"},{"key":"pcbi.1008139.ref079","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1111\/ejh.12021","article-title":"Predictors of hemoglobin response to eculizumab therapy in paroxysmal nocturnal hemoglobinuria","volume":"90","author":"AE DeZern","year":"2013","journal-title":"Eur J Haematol"},{"key":"pcbi.1008139.ref080","first-page":"30","article-title":"PNH is a debilitating, fatal but treatable disease: same disease, different clinical presentations","volume":"5","author":"F Sahin","year":"2015","journal-title":"Am J Blood Res"},{"key":"pcbi.1008139.ref081","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1007\/s00281-011-0280-x","article-title":"Interactions between coagulation and complement\u2014their role in inflammation","volume":"34","author":"K Oikonomopoulou","year":"2012","journal-title":"Semin Immunopathol"},{"key":"pcbi.1008139.ref082","doi-asserted-by":"crossref","first-page":"1235","DOI":"10.1128\/IAI.11.6.1235-1243.1975","article-title":"Function of the classical and alternate pathways of human complement in serum treated with ethylene glycol tetraacetic acid and MgCl2-ethylene glycol tetraacetic acid","volume":"11","author":"RM Des Prez","year":"1975","journal-title":"Infect Immun"},{"key":"pcbi.1008139.ref083","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1128\/IAI.16.1.124-128.1977","article-title":"Comparison of ethyleneglycoltetraacetic acid and its magnesium salt as reagents for studying alternative complement pathway function","volume":"16","author":"DP Fine","year":"1977","journal-title":"Infect Immun"}],"container-title":["PLOS Computational Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pcbi.1008139","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,8]],"date-time":"2023-10-08T23:11:32Z","timestamp":1696806692000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pcbi.1008139"}},"subtitle":[],"editor":[{"given":"James R.","family":"Faeder","sequence":"first","affiliation":[]}],"short-title":[],"issued":{"date-parts":[[2020,10,2]]},"references-count":83,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2020,10,2]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pcbi.1008139","relation":{},"ISSN":["1553-7358"],"issn-type":[{"value":"1553-7358","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,10,2]]}}}