{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T16:57:52Z","timestamp":1787590672017,"version":"build-2736575974"},"reference-count":86,"publisher":"MDPI AG","issue":"22","license":[{"start":{"date-parts":[[2020,11,23]],"date-time":"2020-11-23T00:00:00Z","timestamp":1606089600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"ERA PerMed Call 2018","award":["RC. 2755826"],"award-info":[{"award-number":["RC. 2755826"]}]},{"DOI":"10.13039\/100013276","name":"Interreg","doi-asserted-by":"publisher","award":["Italia-Slovenia, ISE-EMH 07\/2019"],"award-info":[{"award-number":["Italia-Slovenia, ISE-EMH 07\/2019"]}],"id":[{"id":"10.13039\/100013276","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Notch pathway is a highly conserved intracellular signaling route that modulates a vast variety of cellular processes including proliferation, differentiation, migration, cell fate and death. Recently, the presence of a strict crosstalk between Notch signaling and inflammation has been described, although the precise molecular mechanisms underlying this interplay have not yet been fully unravelled. Disruptions in Notch cascade, due both to direct mutations and\/or to an altered regulation in the core components of Notch signaling, might lead to hypo- or hyperactivation of Notch target genes and signaling molecules, ultimately contributing to the onset of autoinflammatory diseases. To date, alterations in Notch signaling have been reported as associated with three autoinflammatory disorders, therefore, suggesting a possible role of Notch in the pathogenesis of the following diseases: hidradenitis suppurativa (HS), Beh\u00e7et disease (BD), and giant cell arteritis (GCA). In this review, we aim at better characterizing the interplay between Notch and autoinflammatory diseases, trying to identify the role of this signaling route in the context of these disorders.<\/jats:p>","DOI":"10.3390\/ijms21228847","type":"journal-article","created":{"date-parts":[[2020,11,23]],"date-time":"2020-11-23T08:18:23Z","timestamp":1606119503000},"page":"8847","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Notch Signaling Regulation in Autoinflammatory Diseases"],"prefix":"10.3390","volume":"21","author":[{"given":"Rossella","family":"Gratton","sequence":"first","affiliation":[{"name":"Institute for Maternal and Child Health\u2014IRCCS \u201cBurlo Garofolo\u201d, 34137 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1954-4126","authenticated-orcid":false,"given":"Paola Maura","family":"Tricarico","sequence":"additional","affiliation":[{"name":"Institute for Maternal and Child Health\u2014IRCCS \u201cBurlo Garofolo\u201d, 34137 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9367-4909","authenticated-orcid":false,"given":"Adamo Pio","family":"d'Adamo","sequence":"additional","affiliation":[{"name":"Institute for Maternal and Child Health\u2014IRCCS \u201cBurlo Garofolo\u201d, 34137 Trieste, Italy"},{"name":"Department of Medical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna Monica","family":"Bianco","sequence":"additional","affiliation":[{"name":"Institute for Maternal and Child Health\u2014IRCCS \u201cBurlo Garofolo\u201d, 34137 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald","family":"Moura","sequence":"additional","affiliation":[{"name":"Institute for Maternal and Child Health\u2014IRCCS \u201cBurlo Garofolo\u201d, 34137 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Almerinda","family":"Agrelli","sequence":"additional","affiliation":[{"name":"Department of Pathology, Federal University of Pernambuco, Recife 50670-901, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucas","family":"Brand\u00e3o","sequence":"additional","affiliation":[{"name":"Institute for Maternal and Child Health\u2014IRCCS \u201cBurlo Garofolo\u201d, 34137 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5886-9129","authenticated-orcid":false,"given":"Luisa","family":"Zupin","sequence":"additional","affiliation":[{"name":"Institute for Maternal and Child Health\u2014IRCCS \u201cBurlo Garofolo\u201d, 34137 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8493-1168","authenticated-orcid":false,"given":"Sergio","family":"Crovella","sequence":"additional","affiliation":[{"name":"Department of Biological and Environmental Sciences, College of Arts and Sciences, University of Qatar, Doha 2713, Qatar"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,11,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1016\/j.bbadis.2008.06.008","article-title":"Notch signaling and diseases: An evolutionary journey from a simple beginning to complex outcomes","volume":"1782","author":"Talora","year":"2008","journal-title":"Biochim. 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