{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,9]],"date-time":"2026-04-09T06:07:22Z","timestamp":1775714842961,"version":"3.50.1"},"reference-count":11,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2018,4,19]],"date-time":"2018-04-19T00:00:00Z","timestamp":1524096000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["AR044232"],"award-info":[{"award-number":["AR044232"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Experimental Dermatology"],"published-print":{"date-parts":[[2018,6]]},"abstract":"Abstract<\/jats:title>The type I intermediate filament keratin 16 (K16) is constitutively expressed in ectoderm\u2010derived appendages and is inducibly expressed in the epidermis upon barrier\u2010compromising challenges. Dominantly acting missense alleles in KRT<\/jats:styled-content>16<\/jats:italic> are causative for pachyonychia congenita (PC<\/jats:styled-content>), a genodermatosis involving debilitating palmoplantar keratoderma (PPK<\/jats:styled-content>), nail dystrophy, oral lesions and, frequently, alterations in glands and hair. C57Bl\/6;Krt16<\/jats:italic>\u2010\/\u2010<\/jats:italic><\/jats:sup> mice develop oral lesions early after birth and PC<\/jats:styled-content>\u2010like PPK<\/jats:styled-content> lesions as young adults. These PPK<\/jats:styled-content> lesions have a marked dysregulation of skin barrier\u2010related genes and innate immunity effectors (eg danger\u2010associated molecular patterns) and are preceded by oxidative stress secondary to hypoactive Nrf2 signalling. These molecular features are present in PPK<\/jats:styled-content> lesions of PC<\/jats:styled-content> patients. Here, we report that all components of the C57Bl\/6;Krt16<\/jats:italic>\u2010\/\u2010<\/jats:italic><\/jats:sup> mouse phenotype occur as well in the FVB<\/jats:styled-content><\/jats:italic> strain background, albeit less severely so, a significant observation in the light of variations in the clinical presentation of individuals harbouring disease\u2010causing mutations in the KRT<\/jats:styled-content>16<\/jats:italic> gene.<\/jats:p>","DOI":"10.1111\/exd.13509","type":"journal-article","created":{"date-parts":[[2018,2,6]],"date-time":"2018-02-06T15:02:42Z","timestamp":1517929362000},"page":"672-674","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["The keratin 16 null phenotype is modestly impacted by genetic strain background in mice"],"prefix":"10.1111","volume":"27","author":[{"given":"Abigail","family":"Zieman","sequence":"first","affiliation":[{"name":"Department of Biochemistry and Molecular Biology Bloomberg School of Public Health Johns Hopkins University Baltimore MD USA"},{"name":"Department of Cell and Developmental Biology University of Michigan Medical School Ann Arbor MI USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0680-2373","authenticated-orcid":false,"given":"Pierre A.","family":"Coulombe","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology Bloomberg School of Public Health Johns Hopkins University Baltimore MD USA"},{"name":"Department of Cell and Developmental Biology University of Michigan Medical School Ann Arbor MI USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,4,19]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"173","volume":"31","author":"McGowan K.","year":"1998","journal-title":"Subcell. Biochem."},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0395-273"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1087-0024.2005.10202.x"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/4.10.1875"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2012.6"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1309576110"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI84870"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.3355"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jid.2017.09.054"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1177\/0300985811429314"},{"key":"e_1_2_6_12_1","doi-asserted-by":"crossref","unstructured":"A.Chaiuduri N. S.Wilson B.Yang A.Paler Martinez J.Liu C.Zhu N.Bricker S.Couto Z.Modrusan D.French J.Cupp A.Ashkenazi Host genetic background impacts modulation of the TLR4 pathway by RON in tissue\u2010associate macrophages.2013 91 451.","DOI":"10.1038\/icb.2013.27"}],"container-title":["Experimental Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fexd.13509","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/exd.13509","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,16]],"date-time":"2023-09-16T20:24:31Z","timestamp":1694895871000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/exd.13509"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,4,19]]},"references-count":11,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2018,6]]}},"alternative-id":["10.1111\/exd.13509"],"URL":"https:\/\/doi.org\/10.1111\/exd.13509","archive":["Portico"],"relation":{},"ISSN":["0906-6705","1600-0625"],"issn-type":[{"value":"0906-6705","type":"print"},{"value":"1600-0625","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,4,19]]},"assertion":[{"value":"2018-01-18","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-04-19","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}