{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T22:43:37Z","timestamp":1789425817847,"version":"build-2803163510"},"reference-count":31,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2019,7,31]],"date-time":"2019-07-31T00:00:00Z","timestamp":1564531200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100002825","name":"Dystrophic Epidermolysis Bullosa Research Association of America","doi-asserted-by":"publisher","award":["Brunton1"],"award-info":[{"award-number":["Brunton1"]}],"id":[{"id":"10.13039\/100002825","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Experimental Dermatology"],"published-print":{"date-parts":[[2019,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Kindler syndrome (KS) is an autosomal recessive skin disorder characterized by skin blistering and photosensitivity. KS is caused by loss of function mutations in <jats:italic>FERMT1<\/jats:italic>, which encodes Kindlin\u20101. Kindlin\u20101 is a FERM domain containing adaptor protein that is found predominantly at cell\u2010extracellular matrix adhesions where it binds to integrin \u03b2 subunits and is required for efficient integrin activation. Using keratinocytes derived from a patient with KS, into which wild\u2010type Kindlin\u20101 (Kin1WT) has been expressed, we show that Kindlin\u20101 binds to cyclin\u2010dependent kinase (CDK)1 and CDK2. CDK1 and CDK2 are key regulators of cell cycle progression, however, cell cycle analysis showed only small differences between the KS and KS\u2010Kin1WT keratinocytes. In contrast, G2\/M cell cycle arrest in response to oxidative stress induced by hydrogen peroxide (H<jats:sub>2<\/jats:sub>O<jats:sub>2<\/jats:sub>) was enhanced in KS keratinocytes but not KS\u2010Kin1WT cells, following inhibition of CDK activity. Furthermore, KS keratinocytes were more sensitive to DNA damage in response to H<jats:sub>2<\/jats:sub>O<jats:sub>2<\/jats:sub> and this was exacerbated by treatment with the CDK inhibitor roscovitine. Thus, in Kindlin\u20101 deficient keratinocytes, CDK activity can further regulate oxidative damage induced cell cycle arrest and DNA damage. This provides further insight into the key pathways that control sensitivity to oxidative stress in KS patients.<\/jats:p>","DOI":"10.1111\/exd.14000","type":"journal-article","created":{"date-parts":[[2019,7,1]],"date-time":"2019-07-01T17:22:53Z","timestamp":1562001773000},"page":"1074-1078","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Inhibition of cyclin\u2010dependent kinase activity exacerbates H<sub>2<\/sub>O<sub>2<\/sub>\u2010induced DNA damage in Kindler syndrome keratinocytes"],"prefix":"10.1111","volume":"28","author":[{"given":"Hila","family":"Emmert","sequence":"first","affiliation":[{"name":"Edinburgh Cancer Research UK Centre, Institute of Genetics &amp; Molecular Medicine University of Edinburgh Edinburgh UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jayne","family":"Culley","sequence":"additional","affiliation":[{"name":"Edinburgh Cancer Research UK Centre, Institute of Genetics &amp; Molecular Medicine University of Edinburgh Edinburgh UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7778-8794","authenticated-orcid":false,"given":"Valerie G.","family":"Brunton","sequence":"additional","affiliation":[{"name":"Edinburgh Cancer Research UK Centre, Institute of Genetics &amp; Molecular Medicine University of Edinburgh Edinburgh UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,7,31]]},"reference":[{"issue":"17","key":"e_1_2_8_2_1","volume":"129","author":"Rognoni E","year":"2016","journal-title":"J. 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