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Skin features, which give their name to the disease and are considered as diagnostic as well as prognostic markers, have not been thoroughly investigated in terms of therapeutic targets. <jats:styled-content style=\"fixed-case\">CCN<\/jats:styled-content> proteins (<jats:styled-content style=\"fixed-case\">CYR<\/jats:styled-content>61\/<jats:styled-content style=\"fixed-case\">CCN<\/jats:styled-content>1, <jats:styled-content style=\"fixed-case\">CTGF<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">CCN<\/jats:styled-content>2, <jats:styled-content style=\"fixed-case\">NOV<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">CCN<\/jats:styled-content>3 and <jats:styled-content style=\"fixed-case\">WISP<\/jats:styled-content>1\u20102\u20103 as <jats:styled-content style=\"fixed-case\">CCN<\/jats:styled-content>4\u20105\u20106) are a family of secreted matricellular proteins implicated in major cellular processes such as cell growth, migration, differentiation. They have already been implicated in key pathophysiological processes of <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c, namely fibrosis, vasculopathy and inflammation. In this review, we discuss the possible implication of <jats:styled-content style=\"fixed-case\">CCN<\/jats:styled-content> proteins in <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c pathogenesis, with a special focus on skin features, and identify the potential actionable <jats:styled-content style=\"fixed-case\">CCN<\/jats:styled-content> targets.<\/jats:p>","DOI":"10.1111\/exd.13806","type":"journal-article","created":{"date-parts":[[2018,10,17]],"date-time":"2018-10-17T13:14:43Z","timestamp":1539782083000},"page":"11-18","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["<scp>CCN<\/scp> proteins as potential actionable targets in scleroderma"],"prefix":"10.1111","volume":"28","author":[{"given":"Pauline","family":"Henrot","sequence":"first","affiliation":[{"name":"University of Bordeaux Inserm BMGIC UMR1035  Bordeaux France"},{"name":"Department of Rheumatology National Reference Center for Rare Diseases Bordeaux University Hospital  Bordeaux France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marie\u2010Elise","family":"Truchetet","sequence":"additional","affiliation":[{"name":"Department of Rheumatology National Reference Center for Rare Diseases Bordeaux University Hospital  Bordeaux France"},{"name":"University of Bordeaux CNRS Immunoconcept UMR 5164  Bordeaux France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gary","family":"Fisher","sequence":"additional","affiliation":[{"name":"Department of Dermatology University of Michigan Medical School  Ann Arbor Michigan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alain","family":"Ta\u00efeb","sequence":"additional","affiliation":[{"name":"University of Bordeaux Inserm BMGIC UMR1035  Bordeaux France"},{"name":"Department of Dermatology University of Michigan Medical School  Ann Arbor Michigan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0462-5684","authenticated-orcid":false,"given":"Muriel","family":"Cario","sequence":"additional","affiliation":[{"name":"University of Bordeaux Inserm BMGIC UMR1035  Bordeaux France"},{"name":"Department of Dermatology and Pediatric Dermatology National Center for Rare Skin Disorders H\u00f4pital Saint Andr\u00e9  Bordeaux France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,12,11]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra0806188"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kep108"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2008.88"},{"key":"e_1_2_13_5_1","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1001\/archderm.1983.01650260037013","volume":"119","author":"S\u00e1nchez J. 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