{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T09:23:47Z","timestamp":1787563427653,"version":"build-2736575974"},"reference-count":53,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2019,1,1]],"date-time":"2019-01-01T00:00:00Z","timestamp":1546300800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acad Dermatol Venereol"],"published-print":{"date-parts":[[2019,4]]},"abstract":"Abstract<\/jats:title>\n \n Background<\/jats:title>\n \n Trappin\u20102\/pre\u2010elafin is an endogenous inhibitor of human neutrophil elastase involved in inflammation, innate immunity and vascular remodelling, which consist of the complex pathological process of systemic sclerosis (\n SS<\/jats:styled-content>\n c).\n <\/jats:p>\n <\/jats:sec>\n \n Objectives<\/jats:title>\n \n To clarify the potential role of trappin\u20102 in\n SS<\/jats:styled-content>\n c.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n Serum trappin\u20102 levels were determined by enzyme\u2010linked immunosorbent assay in 51\n SS<\/jats:styled-content>\n c and 18 healthy subjects. Trappin\u20102 expression was evaluated in\n SS<\/jats:styled-content>\n c lesional skin and cultured endothelial cells treated with\n \n FLI<\/jats:styled-content>\n 1\n <\/jats:italic>\n si\n RNA<\/jats:styled-content>\n by immunohistochemistry, reverse transcription\u2010real\u2010time\n PCR<\/jats:styled-content>\n and\/or immunoblotting. Friend leukaemia virus integration 1 (Fli1) binding to the\n \n PI<\/jats:styled-content>\n 3\n <\/jats:italic>\n promoter was assessed by chromatin immunoprecipitation.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Since serum trappin\u20102 levels inversely correlated with estimated glomerular filtration rate in\n SS<\/jats:styled-content>\n c patients with renal dysfunction,\n SS<\/jats:styled-content>\n c patients with normal renal function were analysed. Although serum trappin\u20102 levels were comparable between diffuse cutaneous\n SS<\/jats:styled-content>\n c, limited cutaneous\n SS<\/jats:styled-content>\n c and control subjects, the prevalence of digital ulcers or elevated right ventricular systolic pressure (\n RVSP<\/jats:styled-content>\n ) was significantly higher in\n SS<\/jats:styled-content>\n c patients with elevated serum trappin\u20102 levels than in those with normal levels. Furthermore, serum trappin\u20102 levels were significantly increased in\n SS<\/jats:styled-content>\n c patients with digital ulcers or elevated\n RVSP<\/jats:styled-content>\n compared to those without. Moreover, serum trappin\u20102 levels positively correlated with\n RVSP<\/jats:styled-content>\n values in\n SS<\/jats:styled-content>\n c patients. Importantly, trappin\u20102 expression was enhanced in small vessels of\n SS<\/jats:styled-content>\n c lesional skin. In cultured endothelial cells, trappin\u20102 expression was elevated by gene silencing of\n \n FLI<\/jats:styled-content>\n 1\n <\/jats:italic>\n at\n mRNA<\/jats:styled-content>\n and protein levels and Fli1 occupied the\n \n PI<\/jats:styled-content>\n 3\n <\/jats:italic>\n promoter.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n \n Endothelial trappin\u20102 up\u2010regulation partially due to Fli1 deficiency can be associated with the development of\n SS<\/jats:styled-content>\n c vasculopathy.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/jdv.15387","type":"journal-article","created":{"date-parts":[[2018,12,6]],"date-time":"2018-12-06T03:01:15Z","timestamp":1544065275000},"page":"753-760","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["A potential contribution of trappin\u20102 to the development of vasculopathy in systemic 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