{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T00:50:57Z","timestamp":1790470257300,"version":"4.1.0"},"reference-count":40,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2019,7,1]],"date-time":"2019-07-01T00:00:00Z","timestamp":1561939200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2019,7,1]],"date-time":"2019-07-01T00:00:00Z","timestamp":1561939200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2023,7,1]],"date-time":"2023-07-01T00:00:00Z","timestamp":1688169600000},"content-version":"vor","delay-in-days":1461,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100007928","name":"Perelman School of Medicine, University of Pennsylvania","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007928","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["AI44970"],"award-info":[{"award-number":["AI44970"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["AI117410"],"award-info":[{"award-number":["AI117410"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["AI085596"],"award-info":[{"award-number":["AI085596"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["EY023709"],"award-info":[{"award-number":["EY023709"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006396","name":"Alexion Pharmaceuticals","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006396","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006458","name":"Children&apos;s Hospital of Philadelphia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006458","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["kidney-international.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Kidney International"],"published-print":{"date-parts":[[2019,7]]},"DOI":"10.1016\/j.kint.2019.01.009","type":"journal-article","created":{"date-parts":[[2019,2,27]],"date-time":"2019-02-27T18:07:47Z","timestamp":1551290867000},"page":"67-79","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":40,"title":["Differential contribution of C5aR and C5b-9 pathways to renal thrombic microangiopathy and macrovascular thrombosis in mice carrying an atypical hemolytic syndrome\u2013related factor H mutation"],"prefix":"10.1016","volume":"96","author":[{"given":"Yoshiyasu","family":"Ueda","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Miwa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daisuke","family":"Ito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hangsoo","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sayaka","family":"Sato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Damodar","family":"Gullipalli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lin","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Madhu","family":"Golla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Delu","family":"Song","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joshua L.","family":"Dunaief","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew B.","family":"Palmer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wen-Chao","family":"Song","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.kint.2019.01.009_bib1","doi-asserted-by":"crossref","first-page":"1676","DOI":"10.1056\/NEJMra0902814","article-title":"Atypical hemolytic-uremic syndrome","volume":"361","author":"Noris","year":"2009","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.kint.2019.01.009_bib2","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/j.molimm.2015.03.246","article-title":"Atypical aHUS: state of the art","volume":"67","author":"Nester","year":"2015","journal-title":"Mol Immunol"},{"key":"10.1016\/j.kint.2019.01.009_bib3","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1146\/annurev.med.59.060106.185110","article-title":"Complement regulatory genes and hemolytic uremic syndromes","volume":"59","author":"Kavanagh","year":"2008","journal-title":"Annu Rev Med"},{"key":"10.1016\/j.kint.2019.01.009_bib4","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1016\/j.semnephrol.2013.08.003","article-title":"Atypical hemolytic uremic syndrome","volume":"33","author":"Kavanagh","year":"2013","journal-title":"Semin Nephrol"},{"key":"10.1016\/j.kint.2019.01.009_bib5","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1086\/318203","article-title":"Factor H mutations in hemolytic uremic syndrome cluster in exons 18-20, a domain important for host cell recognition","volume":"68","author":"Richards","year":"2001","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.kint.2019.01.009_bib6","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1086\/318201","article-title":"Clustering of missense mutations in the C-terminal region of factor H in atypical hemolytic uremic syndrome","volume":"68","author":"Perez-Caballero","year":"2001","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.kint.2019.01.009_bib7","doi-asserted-by":"crossref","first-page":"1542","DOI":"10.1016\/S0140-6736(03)14742-3","article-title":"Familial haemolytic uraemic syndrome and an MCP mutation","volume":"362","author":"Noris","year":"2003","journal-title":"Lancet"},{"key":"10.1016\/j.kint.2019.01.009_bib8","doi-asserted-by":"crossref","first-page":"12966","DOI":"10.1073\/pnas.2135497100","article-title":"Mutations in human complement regulator, membrane cofactor protein (CD46), predispose to development of familial hemolytic uremic syndrome","volume":"100","author":"Richards","year":"2003","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.kint.2019.01.009_bib9","doi-asserted-by":"crossref","first-page":"e84","DOI":"10.1136\/jmg.2004.019083","article-title":"Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome","volume":"41","author":"Fremeaux-Bacchi","year":"2004","journal-title":"J\u00a0Med Genet"},{"key":"10.1016\/j.kint.2019.01.009_bib10","doi-asserted-by":"crossref","first-page":"2150","DOI":"10.1681\/ASN.2005010103","article-title":"Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome","volume":"16","author":"Kavanagh","year":"2005","journal-title":"J\u00a0Am Soc Nephrol"},{"key":"10.1016\/j.kint.2019.01.009_bib11","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1073\/pnas.0603420103","article-title":"Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome","volume":"104","author":"Goicoechea de Jorge","year":"2007","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.kint.2019.01.009_bib12","doi-asserted-by":"crossref","first-page":"4948","DOI":"10.1182\/blood-2008-01-133702","article-title":"Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome","volume":"112","author":"Fremeaux-Bacchi","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.kint.2019.01.009_bib13","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1038\/nrneph.2014.250","article-title":"Podocyte dysfunction in atypical haemolytic uraemic syndrome","volume":"11","author":"Noris","year":"2015","journal-title":"Nat Rev Nephrol"},{"key":"10.1016\/j.kint.2019.01.009_bib14","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1681\/ASN.2004050380","article-title":"Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome","volume":"16","author":"Dragon-Durey","year":"2005","journal-title":"J\u00a0Am Soc Nephrol"},{"key":"10.1016\/j.kint.2019.01.009_bib15","doi-asserted-by":"crossref","first-page":"417","DOI":"10.2215\/CJN.05750611","article-title":"Factor I autoantibodies in patients with atypical hemolytic uremic syndrome: disease-associated or an epiphenomenon?","volume":"7","author":"Kavanagh","year":"2012","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1016\/j.kint.2019.01.009_bib16","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.imlet.2014.01.014","article-title":"Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome","volume":"160","author":"Jozsi","year":"2014","journal-title":"Immunol Lett"},{"key":"10.1016\/j.kint.2019.01.009_bib17","doi-asserted-by":"crossref","first-page":"2701","DOI":"10.1182\/blood-2015-11-680009","article-title":"Disturbed sialic acid recognition on endothelial cells and platelets in complement attack causes atypical hemolytic uremic syndrome","volume":"127","author":"Hyvarinen","year":"2016","journal-title":"Blood"},{"key":"10.1016\/j.kint.2019.01.009_bib18","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1186\/1750-1172-6-60","article-title":"Atypical hemolytic uremic syndrome","volume":"6","author":"Loirat","year":"2011","journal-title":"Orphanet J Rare Dis"},{"key":"10.1016\/j.kint.2019.01.009_bib19","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1016\/j.hoc.2013.02.006","article-title":"Thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome: an update","volume":"27","author":"Tsai","year":"2013","journal-title":"Hematol Oncol Clin North Am"},{"key":"10.1016\/j.kint.2019.01.009_bib20","doi-asserted-by":"crossref","first-page":"34","DOI":"10.5582\/irdr.2014.01001","article-title":"Current treatment of atypical hemolytic uremic syndrome","volume":"3","author":"Kaplan","year":"2014","journal-title":"Intractable Rare Dis Res"},{"key":"10.1016\/j.kint.2019.01.009_bib21","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1038\/cr.2009.139","article-title":"Complement and its role in innate and adaptive immune responses","volume":"20","author":"Dunkelberger","year":"2010","journal-title":"Cell Res"},{"key":"10.1016\/j.kint.2019.01.009_bib22","doi-asserted-by":"crossref","first-page":"e0164646","DOI":"10.1371\/journal.pone.0164646","article-title":"Characterization of pharmacologic and pharmacokinetic properties of CCX168, a potent and selective orally administered complement 5a receptor inhibitor, based on preclinical evaluation and randomized phase 1 clinical study","volume":"11","author":"Bekker","year":"2016","journal-title":"PLoS One"},{"key":"10.1016\/j.kint.2019.01.009_bib23","doi-asserted-by":"crossref","first-page":"1671","DOI":"10.1016\/S0140-6736(02)08560-4","article-title":"Combined kidney and liver transplantation for familial haemolytic uraemic syndrome","volume":"359","author":"Remuzzi","year":"2002","journal-title":"Lancet"},{"key":"10.1016\/j.kint.2019.01.009_bib24","doi-asserted-by":"crossref","first-page":"7009","DOI":"10.4049\/jimmunol.0804031","article-title":"The binding of factor H to a complex of physiological polyanions and C3b on cells is impaired in atypical hemolytic uremic syndrome","volume":"182","author":"Ferreira","year":"2009","journal-title":"J\u00a0Immunol"},{"key":"10.1016\/j.kint.2019.01.009_bib25","doi-asserted-by":"crossref","first-page":"1184","DOI":"10.1182\/blood-2016-07-728253","article-title":"Murine systemic thrombophilia and hemolytic uremic syndrome from a factor H point mutation","volume":"129","author":"Ueda","year":"2017","journal-title":"Blood"},{"key":"10.1016\/j.kint.2019.01.009_bib26","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1038\/nrneph.2012.214","article-title":"Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies","volume":"8","author":"Zuber","year":"2012","journal-title":"Nat Rev Nephrol"},{"key":"10.1016\/j.kint.2019.01.009_bib27","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1111\/j.1751-553X.2011.01380.x","article-title":"ICSH recommendations for identification, diagnostic value, and quantitation of schistocytes","volume":"34","author":"Zini","year":"2012","journal-title":"Int J Lab Hematol"},{"key":"10.1016\/j.kint.2019.01.009_bib28","doi-asserted-by":"crossref","first-page":"3402","DOI":"10.4049\/jimmunol.136.9.3402","article-title":"Complement lysis of U937, a nucleated mammalian cell line in the absence of C9: effect of C9 on C5b-8 mediated cell lysis","volume":"136","author":"Morgan","year":"1986","journal-title":"J\u00a0Immunol"},{"key":"10.1016\/j.kint.2019.01.009_bib29","doi-asserted-by":"crossref","first-page":"4032","DOI":"10.4049\/jimmunol.1103141","article-title":"C5aR expression in a novel GFP reporter gene knockin mouse: implications for the mechanism of action of C5aR signaling in T cell immunity","volume":"188","author":"Dunkelberger","year":"2012","journal-title":"J\u00a0Immunol"},{"key":"10.1016\/j.kint.2019.01.009_bib30","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1161\/01.RES.0000252802.25497.b7","article-title":"Platelets as immune cells: bridging inflammation and cardiovascular disease","volume":"100","author":"von Hundelshausen","year":"2007","journal-title":"Circ Res"},{"key":"10.1016\/j.kint.2019.01.009_bib31","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.blre.2006.06.001","article-title":"Platelet-neutrophil-interactions: linking hemostasis and inflammation","volume":"21","author":"Zarbock","year":"2007","journal-title":"Blood Rev"},{"key":"10.1016\/j.kint.2019.01.009_bib32","doi-asserted-by":"crossref","first-page":"s5","DOI":"10.1161\/ATVBAHA.107.158915","article-title":"Platelets: inflammatory firebugs of vascular walls","volume":"28","author":"May","year":"2008","journal-title":"Arterioscler Thromb Vasc Biol"},{"key":"10.1016\/j.kint.2019.01.009_bib33","doi-asserted-by":"crossref","first-page":"4794","DOI":"10.4049\/jimmunol.177.7.4794","article-title":"A\u00a0novel C5a receptor-tissue factor cross-talk in neutrophils links innate immunity to coagulation pathways","volume":"177","author":"Ritis","year":"2006","journal-title":"J\u00a0Immunol"},{"key":"10.1016\/j.kint.2019.01.009_bib34","doi-asserted-by":"crossref","first-page":"2423","DOI":"10.1182\/blood-2007-01-070631","article-title":"Tissue factor: a link between C5a and neutrophil activation in antiphospholipid antibody induced fetal injury","volume":"110","author":"Redecha","year":"2007","journal-title":"Blood"},{"key":"10.1016\/j.kint.2019.01.009_bib35","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1681\/ASN.2012060570","article-title":"Combination of factor H mutation and properdin deficiency causes severe C3 glomerulonephritis","volume":"24","author":"Lesher","year":"2013","journal-title":"J\u00a0Am Soc Nephrol"},{"key":"10.1016\/j.kint.2019.01.009_bib36","doi-asserted-by":"crossref","first-page":"3869","DOI":"10.4049\/jimmunol.172.6.3869","article-title":"Critical protection from renal ischemia reperfusion injury by CD55 and CD59","volume":"172","author":"Yamada","year":"2004","journal-title":"J\u00a0Immunol"},{"key":"10.1016\/j.kint.2019.01.009_bib37","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/0890-8508(87)90022-3","article-title":"Generation of a monoclonal antibody to mouse C5 application in an ELISA assay for detection of anti-C5 antibodies","volume":"1","author":"Frei","year":"1987","journal-title":"Mol Cell Probes"},{"key":"10.1016\/j.kint.2019.01.009_bib38","doi-asserted-by":"crossref","first-page":"1386","DOI":"10.1016\/j.kint.2016.11.018","article-title":"C5 inhibition prevents renal failure in a mouse model of lethal C3 glomerulopathy","volume":"91","author":"Williams","year":"2017","journal-title":"Kidney Int"},{"key":"10.1016\/j.kint.2019.01.009_bib39","doi-asserted-by":"crossref","first-page":"2886","DOI":"10.4049\/jimmunol.1202536","article-title":"Deletion of Crry and DAF on murine platelets stimulates thrombopoiesis and increases factor H-dependent resistance of peripheral platelets to complement attack","volume":"190","author":"Barata","year":"2013","journal-title":"J\u00a0Immunol"},{"key":"10.1016\/j.kint.2019.01.009_bib40","doi-asserted-by":"crossref","first-page":"4544","DOI":"10.1182\/blood-2002-06-1875","article-title":"Complement-mediated clearance of erythrocytes: mechanism and delineation of the regulatory roles of Crry and DAF: decay-accelerating factor","volume":"100","author":"Molina","year":"2002","journal-title":"Blood"}],"container-title":["Kidney International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S008525381930050X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S008525381930050X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T14:54:06Z","timestamp":1777474446000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S008525381930050X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,7]]},"references-count":40,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2019,7]]}},"alternative-id":["S008525381930050X"],"URL":"https:\/\/doi.org\/10.1016\/j.kint.2019.01.009","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.735408321.793560486","asserted-by":"object"}]},"ISSN":["0085-2538"],"issn-type":[{"value":"0085-2538","type":"print"}],"subject":[],"published":{"date-parts":[[2019,7]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Differential contribution of C5aR and C5b-9 pathways to renal thrombic microangiopathy and macrovascular thrombosis in mice carrying an atypical hemolytic syndrome\u2013related factor H mutation","name":"articletitle","label":"Article Title"},{"value":"Kidney International","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.kint.2019.01.009","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2019 International Society of Nephrology. Published by Elsevier Inc.","name":"copyright","label":"Copyright"}]}}