{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:19:03Z","timestamp":1784557143045,"version":"3.55.0"},"reference-count":50,"publisher":"Bioscientifica","issue":"1","license":[{"start":{"date-parts":[[2022,11,1]],"date-time":"2022-11-01T00:00:00Z","timestamp":1667260800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["vb.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,11,1]]},"abstract":"<jats:p>\n                    Marfan syndrome (MFS) is a connective tissue disorder causing aortic aneurysm formation. Currently, only prophylactic aortic surgery and blood pressure-lowering drugs are available to reduce the risk of aortic rupture. Upon whole genome sequencing of a Marfan family, we identified a complement gene C1R variant (p.Ser152Leu), which is associated with severe aortic patients. Therefore, we assessed the role of complement activation in MFS aortic tissue. Expression of various complement genes and proteins was detected in human and murine MFS aneurysm tissue, which prompted us to study complement inhibition in MFS mice. Treatment of the\n                    <jats:italic>Fbn1<\/jats:italic>\n                    <jats:sup>C1041G\/+<\/jats:sup>\n                    MFS mice with human plasma-derived C1-esterase inhibitor Cetor\u00ae resulted in reduced complement deposition, decreased macrophage influx in the aorta, and lower circulating TNF\u03b1 levels. However, in line with previous anti-inflammatory treatments, complement inhibition did not change the aortic dilatation rate in this MFS mouse model. Thus, while complement factors\/component 3 activation were detected in human\/murine MFS aorta, Cetor\u00ae had no effect on aortic dilatation in MFS mice, indicating that complement inhibition is not a suitable treatment strategy in MFS.\n                  <\/jats:p>","DOI":"10.1530\/vb-22-0016","type":"journal-article","created":{"date-parts":[[2022,10,24]],"date-time":"2022-10-24T12:05:48Z","timestamp":1666613148000},"page":"40-49","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-12","source":"Crossref","is-referenced-by-count":2,"title":["No prominent role for complement C1-esterase inhibitor in Marfan syndrome mice"],"prefix":"10.1530","volume":"4","author":[{"given":"Stijntje","family":"Hibender","sequence":"first","affiliation":[{"name":"Amsterdam UMC Location University of Amsterdam, Department of Medical Biochemistry, Meibergdreef, Amsterdam, The Netherlands"},{"name":"Amsterdam Cardiovascular Sciences, Atherosclerosis & Ischemic Syndromes, Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siyu","family":"Li","sequence":"additional","affiliation":[{"name":"Amsterdam UMC Location University of Amsterdam, Department of Medical Biochemistry, Meibergdreef, Amsterdam, The Netherlands"},{"name":"Amsterdam Cardiovascular Sciences, Atherosclerosis & Ischemic Syndromes, Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alex V","family":"Postma","sequence":"additional","affiliation":[{"name":"Amsterdam Cardiovascular Sciences, Atherosclerosis & Ischemic Syndromes, Amsterdam, The Netherlands"},{"name":"Amsterdam UMC Location University of Amsterdam, Department of Medical Biology, Meibergdreef, Amsterdam, The Netherlands"},{"name":"Amsterdam UMC Location University of Amsterdam, Department of Human Genetics, Meibergdreef, Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Myrthe E","family":"Hoogeland","sequence":"additional","affiliation":[{"name":"Amsterdam UMC Location University of Amsterdam, Department of Medical Biochemistry, Meibergdreef, Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Denise","family":"Klaver","sequence":"additional","affiliation":[{"name":"Amsterdam UMC Location University of Amsterdam, Department of Medical Biochemistry, Meibergdreef, Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard B","family":"Pouw","sequence":"additional","affiliation":[{"name":"Sanquin Research, Department of Immunopathology, Plesmanlaan, Amsterdam, The Netherlands"},{"name":"Amsterdam UMC Location University of Amsterdam, Landsteiner Laboratory, Meibergdreef, Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans W","family":"Niessen","sequence":"additional","affiliation":[{"name":"Amsterdam Cardiovascular Sciences, 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