{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T18:51:21Z","timestamp":1786992681293,"version":"build-2736575974"},"reference-count":45,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2022,4,1]],"date-time":"2022-04-01T00:00:00Z","timestamp":1648771200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2022,4,1]],"date-time":"2022-04-01T00:00:00Z","timestamp":1648771200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2021,9,29]],"date-time":"2021-09-29T00:00:00Z","timestamp":1632873600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["jidonline.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Investigative Dermatology"],"published-print":{"date-parts":[[2022,4]]},"DOI":"10.1016\/j.jid.2021.09.008","type":"journal-article","created":{"date-parts":[[2021,10,2]],"date-time":"2021-10-02T04:42:15Z","timestamp":1633149735000},"page":"1094-1102.e3","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":23,"title":["Systemic Collagen VII Replacement Therapy for Advanced Recessive Dystrophic Epidermolysis Bullosa"],"prefix":"10.1016","volume":"142","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3949-8531","authenticated-orcid":false,"given":"Christine","family":"Gretzmeier","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7424-0013","authenticated-orcid":false,"given":"Didier","family":"Pin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6372-8048","authenticated-orcid":false,"given":"Johannes S.","family":"Kern","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6809-147X","authenticated-orcid":false,"given":"Mei","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6103-7120","authenticated-orcid":false,"given":"David T.","family":"Woodley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8339-9093","authenticated-orcid":false,"given":"Leena","family":"Bruckner-Tuderman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9305-1573","authenticated-orcid":false,"given":"Mark P.","family":"de Souza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4666-2240","authenticated-orcid":false,"given":"Alexander","family":"Nystr\u00f6m","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jid.2021.09.008_bib1","doi-asserted-by":"crossref","first-page":"1450","DOI":"10.1016\/j.jid.2020.10.024","article-title":"Diversity of mechanisms underlying latent TGF-\u03b2 activation in recessive dystrophic epidermolysis bullosa","volume":"141","author":"Akasaka","year":"2021","journal-title":"J\u00a0Invest Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib2","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1007\/BF03401916","article-title":"Basic fibroblast growth factor: a missing link between collagen VII, increased collagenase, and squamous cell carcinoma in recessive dystrophic epidermolysis bullosa","volume":"4","author":"Arbiser","year":"1998","journal-title":"Mol Med"},{"key":"10.1016\/j.jid.2021.09.008_bib3","doi-asserted-by":"crossref","first-page":"1497","DOI":"10.1016\/j.jid.2019.01.011","article-title":"Thrombospondin-1 is a major activator of TGF-\u03b2 signaling in recessive dystrophic epidermolysis bullosa fibroblasts","volume":"139","author":"Atanasova","year":"2019","journal-title":"J\u00a0Invest Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib4","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1038\/s41572-020-0210-0","article-title":"Epidermolysis bullosa","volume":"6","author":"Bardhan","year":"2020","journal-title":"Nat Rev Dis Primers"},{"key":"10.1016\/j.jid.2021.09.008_bib5","doi-asserted-by":"crossref","first-page":"11703","DOI":"10.1038\/ncomms11703","article-title":"Tenascin-C drives persistence of organ fibrosis","volume":"7","author":"Bhattacharyya","year":"2016","journal-title":"Nat Commun"},{"key":"10.1016\/j.jid.2021.09.008_bib6","doi-asserted-by":"crossref","first-page":"100017","DOI":"10.1016\/j.mbplus.2019.100017","article-title":"Generation of rabbit polyclonal human and murine collagen VII monospecific antibodies: a useful tool for dystrophic epidermolysis bullosa therapy studies","volume":"4","author":"Bornert","year":"2019","journal-title":"Matrix Biol Plus"},{"key":"10.1016\/j.jid.2021.09.008_bib7","first-page":"1257","article-title":"Pseudosyndactyly - an inflammatory and fibrotic wound healing disorder in recessive dystrophic epidermolysis bullosa","volume":"13","author":"Breitenbach","year":"2015","journal-title":"J\u00a0Dtsch Dermatol Ges"},{"key":"10.1016\/j.jid.2021.09.008_bib8","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.exer.2018.06.008","article-title":"Collagen VII deficient mice show morphologic and histologic corneal changes that phenotypically mimic human dystrophic epidermolysis bullosa of the eye","volume":"175","author":"Chen","year":"2018","journal-title":"Exp Eye Res"},{"key":"10.1016\/j.jid.2021.09.008_bib9","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/j.det.2009.10.011","article-title":"Type VII collagen: the anchoring fibril protein at fault in dystrophic epidermolysis bullosa","volume":"28","author":"Chung","year":"2010","journal-title":"Dermatol Clin"},{"key":"10.1016\/j.jid.2021.09.008_bib10","doi-asserted-by":"crossref","first-page":"1238","DOI":"10.1111\/bjd.17858","article-title":"Bone marrow transplant with post-transplant cyclophosphamide for recessive dystrophic epidermolysis bullosa expands the related donor pool and permits tolerance of nonhaematopoietic cellular grafts","volume":"181","author":"Ebens","year":"2019","journal-title":"Br J Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib11","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1001\/archderm.1965.01600160118025","article-title":"Cell renewal in human epidermis","volume":"92","author":"Epstein","year":"1965","journal-title":"Arch Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib12","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1016\/j.jaad.2009.03.052","article-title":"Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues","volume":"61","author":"Fine","year":"2009","journal-title":"J\u00a0Am Acad Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib13","doi-asserted-by":"crossref","first-page":"1669","DOI":"10.1172\/JCI34292","article-title":"A\u00a0hypomorphic mouse model of dystrophic epidermolysis bullosa reveals mechanisms of disease and response to fibroblast therapy","volume":"118","author":"Fritsch","year":"2008","journal-title":"J\u00a0Clin Invest"},{"key":"10.1016\/j.jid.2021.09.008_bib14","doi-asserted-by":"crossref","first-page":"2069","DOI":"10.1038\/jid.2011.172","article-title":"Correction of dog dystrophic epidermolysis bullosa by transplantation of genetically modified epidermal autografts","volume":"131","author":"Gache","year":"2011","journal-title":"J\u00a0Invest Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib15","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.matbio.2017.01.002","article-title":"Stromal microenvironment in type VII collagen-deficient skin: the ground for squamous cell carcinoma development","volume":"63","author":"Guerra","year":"2017","journal-title":"Matrix Biol"},{"key":"10.1016\/j.jid.2021.09.008_bib16","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1038\/s41584-019-0324-5","article-title":"Evasion of apoptosis by myofibroblasts: a hallmark of fibrotic diseases","volume":"16","author":"Hinz","year":"2020","journal-title":"Nat Rev Rheumatol"},{"key":"10.1016\/j.jid.2021.09.008_bib17","doi-asserted-by":"crossref","first-page":"1440","DOI":"10.1046\/j.1523-1747.2003.12606.x","article-title":"The organization of human epidermis: functional epidermal units and phi proportionality","volume":"121","author":"Hoath","year":"2003","journal-title":"J\u00a0Invest Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib18","doi-asserted-by":"crossref","first-page":"3060","DOI":"10.1038\/jid.2015.291","article-title":"Intravenously administered recombinant human type VII collagen derived from Chinese hamster ovary cells reverses the disease phenotype in recessive dystrophic epidermolysis bullosa mice","volume":"135","author":"Hou","year":"2015","journal-title":"J\u00a0Invest Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib19","doi-asserted-by":"crossref","first-page":"100065","DOI":"10.1016\/j.mbplus.2021.100065","article-title":"Distinct contributions of meprins to skin regeneration after injury \u2013 meprin \u03b1 a physiological processer of pro-collagen VII","volume":"11","author":"Kruppa","year":"2021","journal-title":"Matrix Biol Plus"},{"key":"10.1016\/j.jid.2021.09.008_bib20","doi-asserted-by":"crossref","first-page":"1116","DOI":"10.1016\/j.jid.2016.02.002","article-title":"Collagen VII half-life at the dermal-epidermal junction zone: implications for mechanisms and therapy of genodermatoses","volume":"136","author":"K\u00fchl","year":"2016","journal-title":"J\u00a0Invest Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib21","doi-asserted-by":"crossref","first-page":"1368","DOI":"10.1038\/mt.2015.58","article-title":"High local concentrations of intradermal MSCs restore skin integrity and facilitate wound healing in dystrophic epidermolysis bullosa","volume":"23","author":"K\u00fchl","year":"2015","journal-title":"Mol Ther"},{"key":"10.1016\/j.jid.2021.09.008_bib22","doi-asserted-by":"crossref","first-page":"2381","DOI":"10.1038\/jid.2014.185","article-title":"Loss of collagen VII is associated with reduced transglutaminase 2 abundance and activity","volume":"134","author":"K\u00fcttner","year":"2014","journal-title":"J\u00a0Invest Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib23","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1038\/msb.2013.17","article-title":"Global remodelling of cellular microenvironment due to loss of collagen VII","volume":"9","author":"K\u00fcttner","year":"2013","journal-title":"Mol Syst Biol"},{"key":"10.1016\/j.jid.2021.09.008_bib24","doi-asserted-by":"crossref","first-page":"1221","DOI":"10.1016\/j.jid.2018.11.036","article-title":"Gene therapy for epidermolysis bullosa","volume":"139","author":"Marinkovich","year":"2019","journal-title":"J\u00a0Invest Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib25","doi-asserted-by":"crossref","first-page":"djv293","DOI":"10.1093\/jnci\/djv293","article-title":"Suppression of TGF\u03b2 and angiogenesis by type VII collagen in cutaneous SCC","volume":"108","author":"Martins","year":"2015","journal-title":"J\u00a0Natl Cancer Inst"},{"key":"10.1016\/j.jid.2021.09.008_bib26","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1007\/s10456-008-9099-z","article-title":"Vascular permeability, vascular hyperpermeability and angiogenesis","volume":"11","author":"Nagy","year":"2008","journal-title":"Angiogenesis"},{"key":"10.1016\/j.jid.2021.09.008_bib27","first-page":"100019","article-title":"Kallikreins: essential epidermal messengers for regulation of the skin microenvironment during homeostasis, repair and disease","volume":"6\u20137","author":"Nauroy","year":"2019","journal-title":"Matrix Biol Plus"},{"key":"10.1016\/j.jid.2021.09.008_bib28","doi-asserted-by":"crossref","first-page":"3522","DOI":"10.1158\/0008-5472.CAN-11-2996","article-title":"Fibroblast-derived dermal matrix drives development of aggressive cutaneous squamous cell carcinoma in patients with recessive dystrophic epidermolysis bullosa","volume":"72","author":"Ng","year":"2012","journal-title":"Cancer Res"},{"key":"10.1016\/j.jid.2021.09.008_bib29","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/j.matbio.2016.07.012","article-title":"Cell therapy for basement membrane-linked diseases","volume":"57\u201358","author":"Nystr\u00f6m","year":"2017","journal-title":"Matrix Biol"},{"key":"10.1016\/j.jid.2021.09.008_bib30","doi-asserted-by":"crossref","first-page":"2035","DOI":"10.1038\/mt.2016.199","article-title":"Gene therapy for epidermolysis bullosa: sticky business","volume":"24","author":"Nystr\u00f6m","year":"2016","journal-title":"Mol Ther"},{"key":"10.1016\/j.jid.2021.09.008_bib31","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1016\/j.matbio.2018.01.016","article-title":"Injury- and inflammation-driven skin fibrosis: the paradigm of epidermolysis bullosa","volume":"68\u201369","author":"Nystr\u00f6m","year":"2018","journal-title":"Matrix Biol"},{"key":"10.1016\/j.jid.2021.09.008_bib32","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.15252\/emmm.201505061","article-title":"Losartan ameliorates dystrophic epidermolysis bullosa and uncovers new disease mechanisms","volume":"7","author":"Nystr\u00f6m","year":"2015","journal-title":"EMBO Mol Med"},{"key":"10.1016\/j.jid.2021.09.008_bib33","doi-asserted-by":"crossref","first-page":"3498","DOI":"10.1172\/JCI68127","article-title":"Collagen VII plays a dual role in wound healing","volume":"123","author":"Nystr\u00f6m","year":"2013","journal-title":"J\u00a0Clin Invest"},{"key":"10.1016\/j.jid.2021.09.008_bib34","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1046\/j.1523-1747.2000.00031-5.x","article-title":"Inherited dystrophic epidermolysis bullosa in inbred dogs: a spontaneous animal model for somatic gene therapy","volume":"115","author":"Palazzi","year":"2000","journal-title":"J\u00a0Invest Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib35","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1016\/j.jaad.2019.11.038","article-title":"Phase I\/II open-label trial of intravenous allogeneic mesenchymal stromal cell therapy in adults with recessive dystrophic epidermolysis bullosa","volume":"83","author":"Rashidghamat","year":"2020","journal-title":"J\u00a0Am Acad Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib36","doi-asserted-by":"crossref","first-page":"e8174","DOI":"10.15252\/msb.20178174","article-title":"Fibroblast state switching orchestrates dermal maturation and wound healing","volume":"14","author":"Rognoni","year":"2018","journal-title":"Mol Syst Biol"},{"key":"10.1016\/j.jid.2021.09.008_bib37","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1074\/mcp.RA117.000437","article-title":"Combinatorial omics analysis reveals perturbed lysosomal homeostasis in collagen VII-deficient keratinocytes","volume":"17","author":"Thriene","year":"2018","journal-title":"Mol Cell Proteomics"},{"key":"10.1016\/j.jid.2021.09.008_bib38","doi-asserted-by":"crossref","first-page":"39027","DOI":"10.1074\/jbc.M006700200","article-title":"Effect of collagen turnover on the accumulation of advanced glycation end products","volume":"275","author":"Verzijl","year":"2000","journal-title":"J\u00a0Biol Chem"},{"key":"10.1016\/j.jid.2021.09.008_bib39","doi-asserted-by":"crossref","first-page":"1335","DOI":"10.1038\/mt.2013.87","article-title":"Topical application of recombinant type VII collagen incorporates into the dermal-epidermal junction and promotes wound closure [published correction appears in Mol Ther 2013;21:11969]","volume":"21","author":"Wang","year":"2013","journal-title":"Mol Ther"},{"key":"10.1016\/j.jid.2021.09.008_bib40","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0137639","article-title":"Lysyl hydroxylase 3 localizes to epidermal basement membrane and is reduced in patients with recessive dystrophic epidermolysis bullosa","volume":"10","author":"Watt","year":"2015","journal-title":"PLoS One"},{"key":"10.1016\/j.jid.2021.09.008_bib41","doi-asserted-by":"crossref","first-page":"693","DOI":"10.1038\/nm1063","article-title":"Injection of recombinant human type VII collagen restores collagen function in dystrophic epidermolysis bullosa","volume":"10","author":"Woodley","year":"2004","journal-title":"Nat Med"},{"key":"10.1016\/j.jid.2021.09.008_bib42","doi-asserted-by":"crossref","first-page":"1575","DOI":"10.1016\/j.jid.2016.03.036","article-title":"Fibroblast-derived MMP-14 regulates collagen homeostasis in adult skin","volume":"136","author":"Zigrino","year":"2016","journal-title":"J\u00a0Invest Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib43","doi-asserted-by":"crossref","first-page":"100017","DOI":"10.1016\/j.mbplus.2019.100017","article-title":"Generation of rabbit polyclonal human and murine collagen VII monospecific antibodies: a useful tool for dystrophic epidermolysis bullosa therapy studies","volume":"4","author":"Bornert","year":"2019","journal-title":"Matrix Biol Plus"},{"key":"10.1016\/j.jid.2021.09.008_bib44","doi-asserted-by":"crossref","first-page":"1116","DOI":"10.1016\/j.jid.2016.02.002","article-title":"Collagen VII half-life at the dermal-epidermal junction zone: implications for mechanisms and therapy of genodermatoses","volume":"136","author":"K\u00fchl","year":"2016","journal-title":"J\u00a0Invest Dermatol"},{"key":"10.1016\/j.jid.2021.09.008_bib45","doi-asserted-by":"crossref","first-page":"1368","DOI":"10.1038\/mt.2015.58","article-title":"High local concentrations of intradermal MSCs restore skin integrity and facilitate wound healing in dystrophic epidermolysis bullosa","volume":"23","author":"K\u00fchl","year":"2015","journal-title":"Mol Ther"}],"container-title":["Journal of Investigative Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0022202X21022739?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0022202X21022739?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,11,3]],"date-time":"2025-11-03T21:48:05Z","timestamp":1762206485000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0022202X21022739"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4]]},"references-count":45,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2022,4]]}},"alternative-id":["S0022202X21022739"],"URL":"https:\/\/doi.org\/10.1016\/j.jid.2021.09.008","relation":{},"ISSN":["0022-202X"],"issn-type":[{"value":"0022-202X","type":"print"}],"subject":[],"published":{"date-parts":[[2022,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Systemic Collagen VII Replacement Therapy for Advanced Recessive Dystrophic Epidermolysis Bullosa","name":"articletitle","label":"Article Title"},{"value":"Journal of Investigative Dermatology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jid.2021.09.008","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2021 The Authors. Published by Elsevier, Inc. on behalf of the Society for Investigative Dermatology.","name":"copyright","label":"Copyright"}]}}