{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T13:42:31Z","timestamp":1791207751222,"version":"4.1.0"},"reference-count":48,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2014,4,14]],"date-time":"2014-04-14T00:00:00Z","timestamp":1397433600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000049","name":"National Institute on Aging","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000049","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005476","name":"Bentley Historical Library, University of Michigan","doi-asserted-by":"publisher","award":["R00HL089413"],"award-info":[{"award-number":["R00HL089413"]}],"id":[{"id":"10.13039\/100005476","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000862","name":"Doris Duke Charitable Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000862","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["faseb.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The FASEB Journal"],"published-print":{"date-parts":[[2014,8]]},"abstract":"<jats:p>\n                    Fibromuscular dysplasia (FMD) is a rare, nonatherosclerotic arterial disease for which the molecular basis is unknown. We comprehensively studied 47 subjects with FMD, including physical examination, spine magnetic resonance imaging, bone densitometry, and brain magnetic resonance angiography. Inflammatory biomarkers in plasma and transforming growth factor \u03b2 (TGF\u2010\u03b2) cytokines in patient\u2010derived dermal fibroblasts were measured by ELISA. Arterial pathology other than medial fibrodysplasia with multifocal stenosis included cerebral aneurysm, found in 12.8% of subjects. Extra\u2010arterial pathology included low bone density (\n                    <jats:italic>P<\/jats:italic>\n                    &lt;0.001); early onset degenerative spine disease (95.7%); increased incidence of Chiari I malformation (6.4%) and dural ectasia (42.6%); and physical examination findings of a mild connective tissue dysplasia (95.7%). Screening for mutations causing known genetically mediated arteriopathies was unrevealing. We found elevated plasma TGF\u2010\u03b21 (\n                    <jats:italic>P<\/jats:italic>\n                    =0.009), TGF\u2010\u03b22\n                    <jats:italic>(P<\/jats:italic>\n                    = 0.004) and additional inflammatory markers, and increased TGF\u2010\u03b21 (\n                    <jats:italic>P<\/jats:italic>\n                    =0.0009) and TGF\u2010\u03b22 (\n                    <jats:italic>P<\/jats:italic>\n                    =0.0001) secretion in dermal fibroblast cell lines from subjects with FMD compared to age\u2010 and gender\u2010matched controls. Detailed phenotyping of patients with FMD allowed us to demonstrate that FMD is a systemic disease with alterations in common with the spectrum of genetic syndromes that involve altered TGF\u2010\u03b2 signaling and offers TGF\u2010\u03b2 as a marker of FMD.\u2014Ganesh, S. K., Morissette, R., Xu, Z., Schoenhoff, F., Griswold, B. F., Yang, J., Tong, L., Yang, M.\u2010L., Hunker, K., Sloper, L., Kuo, S., Raza, R., Milewicz, D. M., Francomano, C. A., Dietz, H. C., Van Eyk, J., McDonnell, N. B. Clinical and biochemical profiles suggest fibromuscular dysplasia is a systemic disease with altered TGF\u2010\u03b2 expression and connective tissue features.\n                    <jats:italic>FASEB J.<\/jats:italic>\n                    28, 3313\u20133324 (2014).\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.fasebj.org\">www.fasebj.org<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1096\/fj.14-251207","type":"journal-article","created":{"date-parts":[[2014,4,15]],"date-time":"2014-04-15T00:06:22Z","timestamp":1397520382000},"page":"3313-3324","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":84,"title":["Clinical and biochemical profiles suggest fibromuscular dysplasia is a systemic disease with altered TGF\u2010\u03b2 expression and connective tissue features"],"prefix":"10.1096","volume":"28","author":[{"given":"Santhi K.","family":"Ganesh","sequence":"first","affiliation":[{"name":"Division of Cardiovascular Medicine, Department of Internal Medicine University of Michigan Ann Arbor Michigan USA"},{"name":"Department of Human Genetics University of Michigan Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachel","family":"Morissette","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Investigation National Institute on Aging Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhi","family":"Xu","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Investigation National Institute on Aging Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florian","family":"Schoenhoff","sequence":"additional","affiliation":[{"name":"Johns Hopkins Bayview Proteomics Center, Department of Medicine Johns Hopkins University Baltimore Maryland 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