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This study was undertaken to determine whether these autoantigens are enriched in cells of the vasculature.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Successive stages of embryoid bodies (EBs) as well as vascular progenitors were used to evaluate the expression of scleroderma autoantigens IFI\u201016 and CENP by immunoblotting. CD31 was included to mark early blood vessels. IFI\u201016 and CD31 expression were defined in paraffin\u2010embedded skin sections from scleroderma patients and from healthy controls. IFI\u201016 expression was determined by flow cytometric analysis in circulating endothelial cells (CECs) and circulating hematopoietic progenitor cells.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Expression of CENP\u2010A, IFI\u201016, and CD31 was enriched in EBs on days 10 and 12 of differentiation, and particularly in cultures enriched in vascular progenitors (IFI\u201016, CD31, and CENPs A and B). This pattern was distinct from that of comparator autoantigens. Immunohistochemical staining of paraffin\u2010embedded skin sections showed enrichment of IFI\u201016 in CD31\u2010positive vascular endothelial cells in biopsy specimens from scleroderma patients and normal controls. Flow cytometric analysis revealed IFI\u201016 expression in circulating hematopoietic progenitor cells but minimal expression in CECs.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our findings indicate that expression of the scleroderma autoantigens IFI\u201016 and CENPs, which are associated with severe vascular disease, is increased in vascular progenitors and mature endothelial cells. High level, lineage\u2010enriched expression of autoantigens may explain the striking association between clinical phenotypes and the immune targeting of specific autoantigens.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.39743","type":"journal-article","created":{"date-parts":[[2016,5,9]],"date-time":"2016-05-09T15:54:33Z","timestamp":1462809273000},"page":"2540-2549","source":"Crossref","is-referenced-by-count":18,"title":["Enrichment of Scleroderma Vascular Disease\u2013Associated Autoantigens in Endothelial Lineage Cells"],"prefix":"10.1002","volume":"68","author":[{"given":"Zsuzsanna H.","family":"McMahan","sequence":"first","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tricia R.","family":"Cottrell","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Baltimore, 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