{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T02:04:53Z","timestamp":1789524293402,"version":"build-2803163510"},"reference-count":67,"publisher":"Elsevier BV","issue":"37","license":[{"start":{"date-parts":[[2011,9,1]],"date-time":"2011-09-01T00:00:00Z","timestamp":1314835200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,9,23]],"date-time":"2020-09-23T00:00:00Z","timestamp":1600819200000},"content-version":"vor","delay-in-days":3310,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Biological Chemistry"],"published-print":{"date-parts":[[2011,9]]},"DOI":"10.1074\/jbc.m111.221804","type":"journal-article","created":{"date-parts":[[2011,7,23]],"date-time":"2011-07-23T01:43:26Z","timestamp":1311385406000},"page":"32810-32823","source":"Crossref","is-referenced-by-count":48,"title":["Classical and Neonatal Marfan Syndrome Mutations in Fibrillin-1 Cause Differential Protease Susceptibilities and Protein Function"],"prefix":"10.1016","volume":"286","author":[{"given":"Ryan","family":"Kirschner","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dirk","family":"Hubmacher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Garud","family":"Iyengar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jasvir","family":"Kaur","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Fagotto-Kaufmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dieter","family":"Br\u00f6mme","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rainer","family":"Bartels","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dieter P.","family":"Reinhardt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1074\/jbc.M111.221804_bib1","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1136\/jmg.2005.039669","volume":"43","author":"Robinson","year":"2006","journal-title":"J. Med. Genet."},{"key":"10.1074\/jbc.M111.221804_bib2","first-page":"23ra20","volume":"2","author":"Loeys","year":"2010","journal-title":"Sc. Transl. Med."},{"key":"10.1074\/jbc.M111.221804_bib3","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1016\/j.ceb.2009.05.005","volume":"21","author":"Ramirez","year":"2009","journal-title":"Curr. Opin. Cell Biol."},{"key":"10.1074\/jbc.M111.221804_bib4","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/S0070-2153(06)75004-9","volume":"75","author":"Hubmacher","year":"2006","journal-title":"Curr. Top. Dev. Biol."},{"key":"10.1074\/jbc.M111.221804_bib5","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1016\/S0065-3233(05)70012-7","volume":"70","author":"Kielty","year":"2005","journal-title":"Adv. Protein Chem."},{"key":"10.1074\/jbc.M111.221804_bib6","doi-asserted-by":"crossref","first-page":"6751","DOI":"10.1074\/jbc.270.12.6751","volume":"270","author":"Handford","year":"1995","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib7","doi-asserted-by":"crossref","first-page":"6659","DOI":"10.1093\/emboj\/16.22.6659","volume":"16","author":"Yuan","year":"1997","journal-title":"EMBO J."},{"key":"10.1074\/jbc.M111.221804_bib8","doi-asserted-by":"crossref","first-page":"12199","DOI":"10.1074\/jbc.M208266200","volume":"278","author":"Smallridge","year":"2003","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib9","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1002\/bdrc.20111","volume":"81","author":"Wagenseil","year":"2007","journal-title":"Birth Defects Res. C. Embryo. Today"},{"key":"10.1074\/jbc.M111.221804_bib10","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1146\/annurev.med.51.1.481","volume":"51","author":"Pyeritz","year":"2000","journal-title":"Annu. Rev. Med."},{"key":"10.1074\/jbc.M111.221804_bib11","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1002\/humu.10249","volume":"22","author":"Collod-B\u00e9roud","year":"2003","journal-title":"Hum. Mutat."},{"key":"10.1074\/jbc.M111.221804_bib12","doi-asserted-by":"crossref","first-page":"51258","DOI":"10.1074\/jbc.M408156200","volume":"279","author":"Suk","year":"2004","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib13","doi-asserted-by":"crossref","first-page":"32924","DOI":"10.1074\/jbc.M405239200","volume":"279","author":"Vollbrandt","year":"2004","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib14","doi-asserted-by":"crossref","first-page":"1231","DOI":"10.1074\/jbc.272.2.1231","volume":"272","author":"Reinhardt","year":"1997","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib15","doi-asserted-by":"crossref","first-page":"1277","DOI":"10.1006\/jmbi.1998.2368","volume":"285","author":"Kettle","year":"1999","journal-title":"J. Mol. Biol."},{"key":"10.1074\/jbc.M111.221804_bib16","doi-asserted-by":"crossref","first-page":"1987","DOI":"10.1093\/hmg\/9.13.1987","volume":"9","author":"McGettrick","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"10.1074\/jbc.M111.221804_bib17","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1038\/ejhg.2008.207","volume":"17","author":"Faivre","year":"2009","journal-title":"Eur. J. Hum. Genet."},{"key":"10.1074\/jbc.M111.221804_bib18","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1038\/ng1116","volume":"33","author":"Neptune","year":"2003","journal-title":"Nat. Genet."},{"key":"10.1074\/jbc.M111.221804_bib19","doi-asserted-by":"crossref","first-page":"2787","DOI":"10.1056\/NEJMoa0706585","volume":"358","author":"Brooke","year":"2008","journal-title":"N. Engl. J. Med."},{"key":"10.1074\/jbc.M111.221804_bib20","doi-asserted-by":"crossref","first-page":"1586","DOI":"10.1172\/JCI200422715","volume":"114","author":"Ng","year":"2004","journal-title":"J. Clin. Invest."},{"key":"10.1074\/jbc.M111.221804_bib21","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1172\/JCI115589","volume":"89","author":"Milewicz","year":"1992","journal-title":"J. Clin. Invest."},{"key":"10.1074\/jbc.M111.221804_bib22","doi-asserted-by":"crossref","first-page":"2135","DOI":"10.1093\/hmg\/2.12.2135","volume":"2","author":"Aoyama","year":"1993","journal-title":"Hum. Mol. Genet."},{"key":"10.1074\/jbc.M111.221804_bib23","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1056\/NEJM199007193230303","volume":"323","author":"Hollister","year":"1990","journal-title":"N. Engl. J. Med."},{"key":"10.1074\/jbc.M111.221804_bib24","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1007\/BF00217450","volume":"90","author":"Raghunath","year":"1993","journal-title":"Hum. Genet."},{"key":"10.1074\/jbc.M111.221804_bib25","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1016\/0945-053X(94)90008-6","volume":"14","author":"Kielty","year":"1994","journal-title":"Matrix Biol."},{"key":"10.1074\/jbc.M111.221804_bib26","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1042\/bj3020889","volume":"302","author":"Raghunath","year":"1994","journal-title":"Biochem. J."},{"key":"10.1074\/jbc.M111.221804_bib27","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/0014-4800(85)90050-4","volume":"43","author":"Halme","year":"1985","journal-title":"Exp. Mol. Pathol."},{"key":"10.1074\/jbc.M111.221804_bib28","doi-asserted-by":"crossref","first-page":"1012","DOI":"10.1016\/S0003-4975(97)00061-1","volume":"63","author":"Fleischer","year":"1997","journal-title":"Ann. Thorac. Surg."},{"key":"10.1074\/jbc.M111.221804_bib29","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1111\/j.1600-0560.1986.tb01515.x","volume":"13","author":"Tsuji","year":"1986","journal-title":"J. Cutan. Pathol."},{"key":"10.1074\/jbc.M111.221804_bib30","doi-asserted-by":"crossref","first-page":"12339","DOI":"10.1074\/jbc.275.16.12339","volume":"275","author":"Reinhardt","year":"2000","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib31","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/S0014-5793(99)00623-7","volume":"452","author":"Hindson","year":"1999","journal-title":"FEBS Lett."},{"key":"10.1074\/jbc.M111.221804_bib32","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1042\/bj3400171","volume":"340","author":"Ashworth","year":"1999","journal-title":"Biochem. J."},{"key":"10.1074\/jbc.M111.221804_bib33","doi-asserted-by":"crossref","first-page":"I365","DOI":"10.1161\/CIRCULATIONAHA.105.000810","volume":"114","author":"Ikonomidis","year":"2006","journal-title":"Circulation"},{"key":"10.1074\/jbc.M111.221804_bib34","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1161\/CIRCRESAHA.107.157776","volume":"101","author":"Chung","year":"2007","journal-title":"Circ. Res."},{"key":"10.1074\/jbc.M111.221804_bib35","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1007\/s00439-004-1194-7","volume":"116","author":"Booms","year":"2005","journal-title":"Hum. Genet."},{"key":"10.1074\/jbc.M111.221804_bib36","first-page":"II331","volume":"98","author":"Segura","year":"1998","journal-title":"Circulation"},{"key":"10.1074\/jbc.M111.221804_bib37","doi-asserted-by":"crossref","first-page":"36761","DOI":"10.1074\/jbc.M403986200","volume":"279","author":"Yasuda","year":"2004","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib38","doi-asserted-by":"crossref","first-page":"2584","DOI":"10.1074\/jbc.M805280200","volume":"284","author":"Wilson","year":"2009","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib39","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1016\/j.atherosclerosis.2007.12.047","volume":"200","author":"Samokhin","year":"2008","journal-title":"Atherosclerosis"},{"key":"10.1074\/jbc.M111.221804_bib40","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1083\/jcb.200608167","volume":"176","author":"Chaudhry","year":"2007","journal-title":"J. Cell Biol."},{"key":"10.1074\/jbc.M111.221804_bib41","doi-asserted-by":"crossref","first-page":"50795","DOI":"10.1074\/jbc.M210611200","volume":"277","author":"Lin","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib42","doi-asserted-by":"crossref","first-page":"39661","DOI":"10.1074\/jbc.M104533200","volume":"276","author":"Jensen","year":"2001","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib43","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1006\/jmbi.1996.0237","volume":"258","author":"Reinhardt","year":"1996","journal-title":"J. Mol. Biol."},{"key":"10.1074\/jbc.M111.221804_bib44","doi-asserted-by":"crossref","first-page":"31854","DOI":"10.1074\/jbc.M602743200","volume":"281","author":"Mellody","year":"2006","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib45","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1021\/bi00504a006","volume":"20","author":"Provencher","year":"1981","journal-title":"Biochemistry"},{"key":"10.1074\/jbc.M111.221804_bib46","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1016\/0003-2697(90)90396-Q","volume":"191","author":"van Stokkum","year":"1990","journal-title":"Anal. Biochem."},{"key":"10.1074\/jbc.M111.221804_bib47","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1002\/pro.5560060421","volume":"6","author":"Linnevers","year":"1997","journal-title":"Protein Sci."},{"key":"10.1074\/jbc.M111.221804_bib48","doi-asserted-by":"crossref","first-page":"2377","DOI":"10.1021\/bi982175f","volume":"38","author":"Br\u00f6mme","year":"1999","journal-title":"Biochemistry"},{"key":"10.1074\/jbc.M111.221804_bib49","doi-asserted-by":"crossref","first-page":"36035","DOI":"10.1074\/jbc.M104985200","volume":"276","author":"Tiedemann","year":"2001","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib50","first-page":"36","volume":"11","author":"Abramoff","year":"2004","journal-title":"Biophoton. Int."},{"key":"10.1074\/jbc.M111.221804_bib51","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1016\/j.ymeth.2004.10.012","volume":"35","author":"Shang","year":"2005","journal-title":"Methods"},{"key":"10.1074\/jbc.M111.221804_bib52","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1006\/jmbi.1998.2536","volume":"286","author":"Smallridge","year":"1999","journal-title":"J. Mol. Biol."},{"key":"10.1074\/jbc.M111.221804_bib53","doi-asserted-by":"crossref","first-page":"1065","DOI":"10.1006\/jmbi.1999.3513","volume":"296","author":"Werner","year":"2000","journal-title":"J. Mol. Biol."},{"key":"10.1074\/jbc.M111.221804_bib54","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1161\/CIRCULATIONAHA.105.561449","volume":"113","author":"Lutgens","year":"2006","journal-title":"Circulation"},{"key":"10.1074\/jbc.M111.221804_bib55","doi-asserted-by":"crossref","first-page":"30526","DOI":"10.1074\/jbc.M501390200","volume":"280","author":"Cain","year":"2005","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib56","doi-asserted-by":"crossref","first-page":"11404","DOI":"10.1074\/jbc.M409882200","volume":"280","author":"Tiedemann","year":"2005","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib57","doi-asserted-by":"crossref","first-page":"6548","DOI":"10.1073\/pnas.0706335105","volume":"105","author":"Hubmacher","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1074\/jbc.M111.221804_bib58","doi-asserted-by":"crossref","first-page":"17156","DOI":"10.1074\/jbc.M006547200","volume":"276","author":"Whiteman","year":"2001","journal-title":"J. Biol. Chem."},{"key":"10.1074\/jbc.M111.221804_bib59","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1007\/0-306-46826-3_30","volume":"477","author":"B\u00fchling","year":"2000","journal-title":"Adv. Exp. Med. Biol."},{"key":"10.1074\/jbc.M111.221804_bib60","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1006\/jmbi.2001.5329","volume":"316","author":"Yuan","year":"2002","journal-title":"J. Mol. Biol."},{"key":"10.1074\/jbc.M111.221804_bib61","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1042\/bj20030649","volume":"375","author":"Ritty","year":"2003","journal-title":"Biochem. J."},{"key":"10.1074\/jbc.M111.221804_bib62","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1006\/geno.1993.1349","volume":"17","author":"Dietz","year":"1993","journal-title":"Genomics"},{"key":"10.1074\/jbc.M111.221804_bib63","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1093\/hmg\/3.2.373","volume":"3","author":"Hayward","year":"1994","journal-title":"Hum. Mol. Genet."},{"key":"10.1074\/jbc.M111.221804_bib64","first-page":"8","volume":"57","author":"Nijbroek","year":"1995","journal-title":"Am. J. Hum. Genet."},{"key":"10.1074\/jbc.M111.221804_bib65","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1046\/j.1440-1754.1999.00355.x","volume":"35","author":"Ng","year":"1999","journal-title":"J. Paediatr. Child Health"},{"key":"10.1074\/jbc.M111.221804_bib66","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1002\/(SICI)1098-1004(1999)13:1<84::AID-HUMU15>3.0.CO;2-U","volume":"13","author":"Perez","year":"1999","journal-title":"Hum. Mutat."},{"key":"10.1074\/jbc.M111.221804_bib67","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1016\/0003-2697(92)90013-W","volume":"207","author":"Brune","year":"1992","journal-title":"Anal. Biochem."}],"container-title":["Journal of Biological Chemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0021925820509885?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0021925820509885?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1074\/jbc.M111.221804","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,1,4]],"date-time":"2022-01-04T22:51:54Z","timestamp":1641336714000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0021925820509885"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,9]]},"references-count":67,"journal-issue":{"issue":"37","published-print":{"date-parts":[[2011,9]]}},"alternative-id":["S0021925820509885"],"URL":"https:\/\/doi.org\/10.1074\/jbc.m111.221804","relation":{},"ISSN":["0021-9258"],"issn-type":[{"value":"0021-9258","type":"print"}],"subject":[],"published":{"date-parts":[[2011,9]]}}}