{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T09:05:09Z","timestamp":1772269509096,"version":"3.50.1"},"reference-count":30,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2013,8,16]],"date-time":"2013-08-16T00:00:00Z","timestamp":1376611200000},"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":[[2013,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Recessive dystrophic epidermolysis bullosa (<jats:styled-content style=\"fixed-case\">RDEB<\/jats:styled-content>) is caused by deficiency of type VII collagen due to <jats:italic><jats:styled-content style=\"fixed-case\">COL<\/jats:styled-content>7A1<\/jats:italic> mutations such as c.6527insC, recurrently found in the <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>panish <jats:styled-content style=\"fixed-case\">RDEB<\/jats:styled-content> population. Assessment of clonal correction\u2013based therapeutic approaches for <jats:styled-content style=\"fixed-case\">RDEB<\/jats:styled-content> requires large expansions of cells, exceeding the replication capacity of human primary keratinocytes. Thus, immortalized <jats:styled-content style=\"fixed-case\">RDEB<\/jats:styled-content> cells with enhanced proliferative abilities would be valuable. Using either the <jats:styled-content style=\"fixed-case\">SV<\/jats:styled-content>40 large <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content> antigen or papillomavirus <jats:styled-content style=\"fixed-case\">HPV<\/jats:styled-content>16\u2010derived E6\u2010E7 proteins, we immortalized and cloned <jats:styled-content style=\"fixed-case\">RDEB<\/jats:styled-content> keratinocytes carrying the c.6527insC mutation. Clones exhibited high proliferative and colony\u2010forming features. Cytogenetic analysis revealed important differences between <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content> antigen\u2010driven and <jats:styled-content style=\"fixed-case\">E<\/jats:styled-content>6\u2010<jats:styled-content style=\"fixed-case\">E<\/jats:styled-content>7\u2010driven immortalization. Immortalized cells responded to differentiation stimuli and were competent for epidermal regeneration and recapitulation of the blistering <jats:styled-content style=\"fixed-case\">RDEB<\/jats:styled-content> phenotype <jats:italic>in vivo<\/jats:italic>. These features make these cell lines useful to test novel therapeutic approaches including those aimed at editing mutant <jats:italic><jats:styled-content style=\"fixed-case\">COL<\/jats:styled-content>7A1<\/jats:italic>.<\/jats:p>","DOI":"10.1111\/exd.12203","type":"journal-article","created":{"date-parts":[[2013,6,28]],"date-time":"2013-06-28T03:43:12Z","timestamp":1372390992000},"page":"601-603","source":"Crossref","is-referenced-by-count":23,"title":["Keratinocyte cell lines derived from severe generalized recessive <scp>E<\/scp>pidermolysis <scp>B<\/scp>ullosa patients carrying a highly recurrent <i><scp>COL<\/scp>7A1<\/i> homozygous mutation: models to assess cell and gene therapies <i>in vitro<\/i> and <i>in vivo<\/i>"],"prefix":"10.1111","volume":"22","author":[{"given":"Cristina","family":"Chamorro","sequence":"first","affiliation":[{"name":"Epithelial Biomedicine Division Cutaneous Disease Modelling Unit CIEMAT Madrid 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