{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T17:38:32Z","timestamp":1793813912215,"version":"4.6.1"},"reference-count":32,"publisher":"Public Library of Science (PLoS)","issue":"3","license":[{"start":{"date-parts":[[2012,3,30]],"date-time":"2012-03-30T00:00:00Z","timestamp":1333065600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0032963","type":"journal-article","created":{"date-parts":[[2012,3,30]],"date-time":"2012-03-30T21:08:49Z","timestamp":1333141729000},"page":"e32963","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":70,"title":["Inflammation Aggravates Disease Severity in Marfan Syndrome Patients"],"prefix":"10.1371","volume":"7","author":[{"given":"Teodora","family":"Radonic","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piet","family":"de Witte","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maarten","family":"Groenink","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vivian","family":"de Waard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rene","family":"Lutter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"van Eijk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marnix","family":"Jansen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janneke","family":"Timmermans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marlies","family":"Kempers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arthur J.","family":"Scholte","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yvonne","family":"Hilhorst-Hofstee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maarten P.","family":"van den Berg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Peter","family":"van Tintelen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerard","family":"Pals","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marieke J. H.","family":"Baars","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara J. M.","family":"Mulder","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aeilko H.","family":"Zwinderman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2012,3,30]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"1965","DOI":"10.1016\/S0140-6736(05)67789-6","article-title":"Marfan&apos;s syndrome.","volume":"366","author":"DP Judge","year":"2005","journal-title":"Lancet"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1038\/352337a0","article-title":"Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene.","volume":"352","author":"HC Dietz","year":"1991","journal-title":"Nature"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1002\/jcp.21189","article-title":"Fibrillin-rich microfibrils: Structural determinants of morphogenetic and homeostatic events.","volume":"213","author":"F Ramirez","year":"2007","journal-title":"J Cell Physiol"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1038\/ng1116","article-title":"Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome.","volume":"33","author":"ER Neptune","year":"2003","journal-title":"Nat Genet"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1126\/science.1192152","article-title":"Angiotensin II type 2 receptor signaling attenuates aortic aneurysm in mice through ERK antagonism.","volume":"332","author":"JP Habashi","year":"2011","journal-title":"Science"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1126\/science.1192149","article-title":"Noncanonical TGFbeta signaling contributes to aortic aneurysm progression in Marfan syndrome mice.","volume":"332","author":"TM Holm","year":"2011","journal-title":"Science"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1038\/nm1536","article-title":"Angiotensin II type 1 receptor blockade attenuates TGF-beta-induced failure of muscle regeneration in multiple myopathic states.","volume":"13","author":"RD Cohn","year":"2007","journal-title":"Nat Med"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1126\/science.1124287","article-title":"Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome.","volume":"312","author":"JP Habashi","year":"2006","journal-title":"Science"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"1586","DOI":"10.1172\/JCI200422715","article-title":"TGF-beta-dependent pathogenesis of mitral valve prolapse in a mouse model of Marfan syndrome.","volume":"114","author":"CM Ng","year":"2004","journal-title":"J Clin Invest"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1172\/JCI38136","article-title":"TGF-beta activity protects against inflammatory aortic aneurysm progression and complications in angiotensin II-infused mice.","volume":"120","author":"Y Wang","year":"2010","journal-title":"J Clin Invest"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"1855","DOI":"10.1161\/CIRCULATIONAHA.105.601674","article-title":"Induction of macrophage chemotaxis by aortic extracts of the mgR Marfan mouse model and a GxxPG-containing fibrillin-1 fragment.","volume":"114","author":"G Guo","year":"2006","journal-title":"Circulation"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"e20138","DOI":"10.1371\/journal.pone.0020138","article-title":"Induction of macrophage chemotaxis by aortic extracts from patients with marfan syndrome is related to elastin binding protein.","volume":"6","author":"G Guo","year":"2011","journal-title":"PLoS One"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1016\/j.jtcvs.2007.12.063","article-title":"Characterization of the inflammatory cells in ascending thoracic aortic aneurysms in patients with Marfan syndrome, familial thoracic aortic aneurysms, and sporadic aneurysms.","volume":"136","author":"R He","year":"2008","journal-title":"J Thorac Cardiovasc Surg"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1111\/j.1399-0004.2007.00845.x","article-title":"Utility of molecular analyses in the exploration of extreme intrafamilial variability in the Marfan syndrome.","volume":"72","author":"J De Backer","year":"2007","journal-title":"Clin Genet"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1086\/520125","article-title":"Effect of mutation type and location on clinical outcome in 1,013 probands with Marfan syndrome or related phenotypes and FBN1 mutations: an international study.","volume":"81","author":"L Faivre","year":"2007","journal-title":"Am J Hum Genet"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"333","DOI":"10.2741\/3247","article-title":"Natural Tregs and autoimmunity.","volume":"14","author":"A La Cava","year":"2009","journal-title":"Front Biosci"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"1722","DOI":"10.1126\/science.1168988","article-title":"CD24 and Siglec-10 selectively repress tissue damage-induced immune responses.","volume":"323","author":"GY Chen","year":"2009","journal-title":"Science"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"3219","DOI":"10.1073\/pnas.0813400106","article-title":"Massively parallel sequencing identifies the gene Megf8 with ENU-induced mutation causing heterotaxy.","volume":"106","author":"Z Zhang","year":"2009","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1016\/j.jvs.2006.09.067","article-title":"The role of human leukocyte antigen genes in the formation of abdominal aortic aneurysms.","volume":"45","author":"SA Badger","year":"2007","journal-title":"J Vasc Surg"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1016\/S0741-5214(97)70358-6","article-title":"Genetic risk factors in inflammatory abdominal aortic aneurysms: polymorphic residue 70 in the HLA-DR B1 gene as a key genetic element.","volume":"25","author":"TE Rasmussen","year":"1997","journal-title":"J Vasc Surg"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1111\/j.1749-6632.1996.tb33311.x","article-title":"A genetic basis for autoimmune manifestations in the abdominal aortic aneurysm resides in the MHC class II locus DR-beta-1.","volume":"800","author":"MD Tilson","year":"1996","journal-title":"Ann N Y Acad Sci"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1002\/art.21698","article-title":"Chronic periaortitis and HLA-DRB1*03: another clue to an autoimmune origin.","volume":"55","author":"D Martorana","year":"2006","journal-title":"Arthritis Rheum"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"738","DOI":"10.1161\/HYPERTENSIONAHA.109.132886","article-title":"Chronic treatment with losartan results in sufficient serum levels of the metabolite EXP3179 for PPARgamma activation.","volume":"54","author":"K Kappert","year":"2009","journal-title":"Hypertension"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1161\/01.RES.0000014434.48463.35","article-title":"Angiotensin II receptor-independent antiinflammatory and antiaggregatory properties of losartan: role of the active metabolite EXP3179.","volume":"90","author":"C Kramer","year":"2002","journal-title":"Circ Res"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"1308","DOI":"10.1001\/archderm.1984.01650460048018","article-title":"Neutrophil chemotaxis in patients with acne receiving oral tetracycline therapy.","volume":"120","author":"NB Esterly","year":"1984","journal-title":"Arch Dermatol"},{"key":"ref26","first-page":"131","article-title":"The influence of divalent cations and doxycycline on iodoacetamide-inhibitable leukocyte adherence.","volume":"74","author":"WL Gabler","year":"1991","journal-title":"Res Commun Chem Pathol Pharmacol"},{"key":"ref27","first-page":"443","article-title":"Inhibition of mitogen-induced human lymphocyte proliferative responses by tetracycline analogues.","volume":"35","author":"YH Thong","year":"1979","journal-title":"Clin Exp Immunol"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"862","DOI":"10.1073\/pnas.0910312107","article-title":"Distinct defects in collagen microarchitecture underlie vessel-wall failure in advanced abdominal aneurysms and aneurysms in Marfan syndrome.","volume":"107","author":"JH Lindeman","year":"2010","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1186\/1745-6215-11-3","article-title":"Losartan therapy in adults with Marfan syndrome: study protocol of the multi-center randomized controlled COMPARE trial.","volume":"11","author":"T Radonic","year":"2010","journal-title":"Trials"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1002\/(SICI)1096-8628(19960424)62:4<417::AID-AJMG15>3.0.CO;2-R","article-title":"Revised diagnostic criteria for the Marfan syndrome.","volume":"62","author":"A De Paepe","year":"1996","journal-title":"Am J Med Genet"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1016\/S0002-9149(98)00315-4","article-title":"Changes in aortic distensibility and pulse wave velocity assessed with magnetic resonance imaging following beta-blocker therapy in the Marfan syndrome.","volume":"82","author":"M Groenink","year":"1998","journal-title":"Am J Cardiol"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"5116","DOI":"10.1073\/pnas.091062498","article-title":"Significance analysis of microarrays applied to the ionizing radiation response.","volume":"98","author":"VG Tusher","year":"2001","journal-title":"Proc Natl Acad Sci U S A"}],"container-title":["PLoS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0032963","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2018,10,19]],"date-time":"2018-10-19T23:57:05Z","timestamp":1539993425000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0032963"}},"subtitle":[],"editor":[{"given":"Sunil K.","family":"Ahuja","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2012,3,30]]},"references-count":32,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2012,3,30]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0032963","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,3,30]]}}}