{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T15:08:33Z","timestamp":1781104113002,"version":"3.54.1"},"reference-count":39,"publisher":"National Academy of Sciences","issue":"5","license":[{"start":{"date-parts":[[2015,7,20]],"date-time":"2015-07-20T00:00:00Z","timestamp":1437350400000},"content-version":"vor","delay-in-days":181,"URL":"http:\/\/www.pnas.org\/site\/misc\/userlicense.xhtml"}],"funder":[{"DOI":"10.13039\/501100005010","name":"Associazione Italiana per la Ricerca sul Cancro","doi-asserted-by":"publisher","award":["IG 13537"],"award-info":[{"award-number":["IG 13537"]}],"id":[{"id":"10.13039\/501100005010","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004410","name":"European Molecular Biology Organization","doi-asserted-by":"publisher","award":["ASTF 15-2008"],"award-info":[{"award-number":["ASTF 15-2008"]}],"id":[{"id":"10.13039\/100004410","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. 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The defect is observed in TTD and not in XP and is specific for fibroblasts, which are the main producers of dermal ECM.\n                    <jats:italic toggle=\"yes\">MMP-1<\/jats:italic>\n                    transcriptional up-regulation in TTD is caused by an erroneous signaling mediated by retinoic acid receptors on the\n                    <jats:italic toggle=\"yes\">MMP-1<\/jats:italic>\n                    promoter and leads to hypersecretion of active MMP-1 enzyme and degradation of collagen type I in the ECM of cell\/tissue systems and TTD patient skin. In agreement with the well-known role of ECM in eliciting signaling events controlling cell behavior and tissue homeostasis, ECM alterations in TTD were shown to impact on the migration and wound-healing properties of patient dermal fibroblasts. The presence of a specific inhibitor of MMP activity was sufficient to restore normal cell migration, thus providing a potential approach for therapeutic strategies. This study highlights the relevance of ECM anomalies in TTD pathogenesis and in the phenotypic differences between TTD and XP.\n                  <\/jats:p>","DOI":"10.1073\/pnas.1416181112","type":"journal-article","created":{"date-parts":[[2015,1,21]],"date-time":"2015-01-21T01:19:22Z","timestamp":1421803162000},"page":"1499-1504","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":28,"title":["TFIIH-dependent\n                    <i>MMP-1<\/i>\n                    overexpression in trichothiodystrophy leads to extracellular matrix alterations in patient skin"],"prefix":"10.1073","volume":"112","author":[{"given":"Lavinia","family":"Arseni","sequence":"first","affiliation":[{"name":"Istituto di Genetica Molecolare Consiglio Nazionale delle Ricerche","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuela","family":"Lanzafame","sequence":"additional","affiliation":[{"name":"Istituto di Genetica 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