{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T05:09:27Z","timestamp":1790744967229,"version":"4.1.0"},"reference-count":17,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2013,3,26]],"date-time":"2013-03-26T00:00:00Z","timestamp":1364256000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Experimental Dermatology"],"published-print":{"date-parts":[[2013,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>An important characteristic of epidermolysis bullosa simplex <jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>owling\u2013<jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>eara (<jats:styled-content style=\"fixed-case\">EBS\u2010DM<\/jats:styled-content>) keratinocytes is the increased level of Jun <jats:styled-content style=\"fixed-case\">N<\/jats:styled-content>\u2010terminal kinase (<jats:styled-content style=\"fixed-case\">JNK<\/jats:styled-content>) stress signalling, which is thought to contribute to the disease phenotype. In this work, we report on the dramatic up\u2010regulation of cytokeratin 14 (<jats:styled-content style=\"fixed-case\">K<\/jats:styled-content>14) in the <jats:styled-content style=\"fixed-case\">EBS\u2010DM<\/jats:styled-content> model cell line <jats:styled-content style=\"fixed-case\">KEB<\/jats:styled-content>7 at both the transcriptional and translational levels, which is noteworthy because <jats:styled-content style=\"fixed-case\">KEB<\/jats:styled-content>7 patient cells are heterozygous for a missense mutation (<jats:styled-content style=\"fixed-case\">R<\/jats:styled-content>125<jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>) in <jats:styled-content style=\"fixed-case\">K<\/jats:styled-content>14. By performing functional assays, we show a direct link between overexpressed wild\u2010type <jats:styled-content style=\"fixed-case\">K<\/jats:styled-content>14 and increased <jats:styled-content style=\"fixed-case\">JNK<\/jats:styled-content> signalling in healthy, immortalized keratinocytes. This observation led us to hypothesize a positive feedback model in which mutant (<jats:styled-content style=\"fixed-case\">R<\/jats:styled-content>125<jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>) <jats:styled-content style=\"fixed-case\">K<\/jats:styled-content>14 triggers <jats:styled-content style=\"fixed-case\">JNK<\/jats:styled-content> signalling, leading to increased <jats:styled-content style=\"fixed-case\">AP<\/jats:styled-content>1\u2010dependent expression of <jats:styled-content style=\"fixed-case\">K<\/jats:styled-content>14, which in turn amplifies <jats:styled-content style=\"fixed-case\">JNK<\/jats:styled-content> signalling further. We therefore suggest that an imbalance of cytoplasmic <jats:styled-content style=\"fixed-case\">K<\/jats:styled-content>14 monomers and <jats:styled-content style=\"fixed-case\">K<\/jats:styled-content>14 incorporated into the intermediate filament (<jats:styled-content style=\"fixed-case\">IF<\/jats:styled-content>) network leads to elevated stress signalling, potentially altering <jats:styled-content style=\"fixed-case\">IF<\/jats:styled-content> dynamics by phosphorylation, which as a side effect, weakens <jats:styled-content style=\"fixed-case\">EBS\u2010DM<\/jats:styled-content> keratinocytes.<\/jats:p>","DOI":"10.1111\/exd.12112","type":"journal-article","created":{"date-parts":[[2013,2,11]],"date-time":"2013-02-11T07:50:42Z","timestamp":1360569042000},"page":"292-294","source":"Crossref","is-referenced-by-count":16,"title":["Imbalance of intermediate filament component keratin 14 contributes to increased stress signalling in epidermolysis bullosa simplex"],"prefix":"10.1111","volume":"22","author":[{"given":"Martin","family":"Wagner","sequence":"first","affiliation":[{"name":"Division of Molecular Dermatology Department of Dermatology Paracelsus Medical University Salzburg Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Trost","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology and Optometry Paracelsus Medical University Salzburg Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helmut","family":"Hintner","sequence":"additional","affiliation":[{"name":"Division of Molecular Dermatology Department of Dermatology Paracelsus Medical University Salzburg Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johann W.","family":"Bauer","sequence":"additional","affiliation":[{"name":"Division of Molecular Dermatology Department of Dermatology Paracelsus Medical University Salzburg Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kamil","family":"Onder","sequence":"additional","affiliation":[{"name":"Division of Molecular Dermatology Department of Dermatology Paracelsus Medical University Salzburg Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,3,26]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(80)90094-X"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00418-008-0435-6"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.1643"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra040319"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12491885"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2011.93"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.00120"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yexcr.2008.02.020"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2009.327"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddi443"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.2003.05493.x"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0625.2011.01420.x"},{"key":"e_1_2_5_14_1","first-page":"361","volume":"6","author":"Ma S","year":"1997","journal-title":"Gene Expr"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200703174"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.jid.5700918"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yexcr.2009.07.013"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0625.2012.01534.x"}],"container-title":["Experimental Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fexd.12112","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/exd.12112","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T22:42:45Z","timestamp":1698705765000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/exd.12112"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,3,26]]},"references-count":17,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2013,4]]}},"alternative-id":["10.1111\/exd.12112"],"URL":"https:\/\/doi.org\/10.1111\/exd.12112","archive":["Portico"],"relation":{},"ISSN":["0906-6705","1600-0625"],"issn-type":[{"value":"0906-6705","type":"print"},{"value":"1600-0625","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,3,26]]}}}