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When mutated, the gene for fibrillin\u20101 (<jats:italic>FBN1<\/jats:italic>) causes the Marfan syndrome, a common inherited disorder of connective tissue. The multiple manifestations of the Marfan syndrome reflect the known distribution of fibrillin\u20101 in cardiovascular, musculoskeletal, ocular, and dermal tissues. In this study, a mouse model of Marfan syndrome in which fibrillin\u20101 is truncated and tagged with green fluorescence was used to estimate the relative abundance of fibrillin\u20101 in developing tissues. In embryonic tissues, the aorta was the only tissue in which fibrillin\u20101 green fluorescence was detectable. Other arteries gained detectable fibrillin\u20101 green fluorescence just after birth. Fibrillin\u20101 fluorescence was observed at later postnatal times in the lung, skin, perichondrium, tendon, and ocular tissues, while other tissues remained negative. These results indicated that tissues most affected in the Marfan syndrome are the tissues in which fibrillin\u20101 is most abundant. Focus was placed on the aorta, since aortic disease is life threatening in the Marfan syndrome and fibrillin\u20101 green fluorescence was most abundant in this tissue. Fibrillin\u20101 green fluorescence and immunostaining showed that fibrillin\u20101 is within aortic medial elastic lamellae. Endothelial\u2010specific compared to smooth muscle\u2010specific fibrillin\u20101 green fluorescence, together with light microscopic analyses of fragmentation of aortic elastic lamellae, demonstrated that smooth muscle cell mutated fibrillin\u20101 contributed most to progressive aortic fragmentation. However, these studies also indicated that other cells, possibly endothelial cells, also contribute to this aortic pathology. Anat Rec, 2019. \u00a9 2019 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/ar.24217","type":"journal-article","created":{"date-parts":[[2019,6,28]],"date-time":"2019-06-28T12:18:24Z","timestamp":1561724304000},"page":"1590-1603","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Fibrillin\u20101 in the Vasculature: <i>In Vivo<\/i> Accumulation of eGFP\u2010Tagged Fibrillin\u20101 in a Knockin Mouse Model"],"prefix":"10.1002","volume":"303","author":[{"given":"Noe L.","family":"Charbonneau","sequence":"first","affiliation":[{"name":"Shriners Hospital for Children Oregon Health and Science University  Oregon Portland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elise C.","family":"Manalo","sequence":"additional","affiliation":[{"name":"Shriners Hospital for Children Oregon Health and Science University  Oregon Portland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sara F.","family":"Tufa","sequence":"additional","affiliation":[{"name":"Shriners Hospital for Children Oregon Health and Science University  Oregon Portland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eric J.","family":"Carlson","sequence":"additional","affiliation":[{"name":"Shriners Hospital for Children Oregon Health and Science University  Oregon Portland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Valerie M.","family":"Carlberg","sequence":"additional","affiliation":[{"name":"Shriners Hospital for Children Oregon Health and Science University  Oregon Portland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Douglas R.","family":"Keene","sequence":"additional","affiliation":[{"name":"Shriners Hospital for Children Oregon Health and Science University  Oregon Portland"},{"name":"Department of Molecular and Medical Genetics Oregon Health and Science University  Oregon 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