{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:25:40Z","timestamp":1791296740110,"version":"4.1.0"},"reference-count":53,"publisher":"Rockefeller University Press","issue":"5","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2001,11,26]]},"abstract":"<jats:p>The desmosomal cadherin desmocollin (Dsc)1 is expressed in upper epidermis where strong adhesion is required. To investigate its role in vivo, we have genetically engineered mice with a targeted disruption in the Dsc1 gene. Soon after birth, null mice exhibit flaky skin and a striking punctate epidermal barrier defect. The epidermis is fragile, and acantholysis in the granular layer generates localized lesions, compromising skin barrier function. Neutrophils accumulate in the lesions and further degrade the tissue, causing sloughing (flaking) of lesional epidermis, but rapid wound healing prevents the formation of overt lesions. Null epidermis is hyperproliferative and overexpresses keratins 6 and 16, indicating abnormal differentiation. From 6 wk, null mice develop ulcerating lesions resembling chronic dermatitis. We speculate that ulceration occurs after acantholysis in the fragile epidermis because environmental insults are more stringent and wound healing is less rapid than in neonatal mice. This dermatitis is accompanied by localized hair loss associated with formation of utriculi and dermal cysts, denoting hair follicle degeneration. Possible resemblance of the lesions to human blistering diseases is discussed. These results show that Dsc1 is required for strong adhesion and barrier maintenance in epidermis and contributes to epidermal differentiation.<\/jats:p>","DOI":"10.1083\/jcb.200105009","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T16:46:23Z","timestamp":1027701983000},"page":"821-832","update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":169,"title":["Mice lacking desmocollin 1 show epidermal fragility accompanied by barrier defects and abnormal differentiation"],"prefix":"10.1083","volume":"155","author":[{"given":"Martyn","family":"Chidgey","sequence":"first","affiliation":[{"name":"1School of Biological Sciences, University of Manchester, Manchester M13 9PT, UK"},{"name":"2Division of Medical Sciences, University of Birmingham, Birmingham B15 2TH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cord","family":"Brakebusch","sequence":"additional","affiliation":[{"name":"3Department of Experimental Pathology, Lund University, S-221 85 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erika","family":"Gustafsson","sequence":"additional","affiliation":[{"name":"3Department of Experimental Pathology, Lund University, S-221 85 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan","family":"Cruchley","sequence":"additional","affiliation":[{"name":"4Clinical and Diagnostic Oral Sciences, Barts and The London, Queen Mary's School of Medicine and Dentistry, London E1 2AT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chris","family":"Hail","sequence":"additional","affiliation":[{"name":"2Division of Medical Sciences, University of Birmingham, Birmingham B15 2TH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Kirk","sequence":"additional","affiliation":[{"name":"1School of Biological Sciences, University of Manchester, Manchester M13 9PT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anita","family":"Merritt","sequence":"additional","affiliation":[{"name":"1School of Biological Sciences, University of Manchester, Manchester M13 9PT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alison","family":"North","sequence":"additional","affiliation":[{"name":"1School of Biological Sciences, University of Manchester, Manchester M13 9PT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chris","family":"Tselepis","sequence":"additional","affiliation":[{"name":"2Division of Medical Sciences, University of Birmingham, Birmingham B15 2TH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jane","family":"Hewitt","sequence":"additional","affiliation":[{"name":"1School of Biological Sciences, University of Manchester, Manchester M13 9PT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolyn","family":"Byrne","sequence":"additional","affiliation":[{"name":"1School of Biological Sciences, University of Manchester, Manchester M13 9PT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reinhard","family":"Fassler","sequence":"additional","affiliation":[{"name":"5Department of Molecular Medicine, Max Plank Institute for Biochemistry, D-82152 Martinsried, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Garrod","sequence":"additional","affiliation":[{"name":"1School of Biological Sciences, University of Manchester, Manchester M13 9PT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2001,11,19]]},"reference":[{"key":"2023072904365757900_BIB1","doi-asserted-by":"crossref","first-page":"1367","DOI":"10.1083\/jcb.133.6.1367","volume":"133","year":"1996","journal-title":"J. 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