{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T18:44:05Z","timestamp":1793213045756,"version":"4.5.0"},"reference-count":47,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2007,5,1]],"date-time":"2007-05-01T00:00:00Z","timestamp":1177977600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2007,5,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Epidermolysis bullosa acquisita is a subepidermal blistering disease associated with tissue-bound and circulating autoantibodies against type VII collagen, a major constituent of the dermal-epidermal junction. The passive transfer of Abs against type VII collagen into mice induces a subepidermal blistering disease dependent upon activation of terminal complement components. To further dissect the role of the different complement activation pathways in this model, we injected C1q-deficient, mannan-binding lectin-deficient, and factor B-deficient mice with rabbit Abs against murine type VII collagen. The development and evolution of blistering had a similar pattern in mannan-binding lectin-deficient and control mice and was initially only marginally less extensive in C1q-deficient mice compared with controls. Importantly, factor B-deficient mice developed a delayed and significantly less severe blistering disease compared with factor B-sufficient mice. A significantly lower neutrophilic infiltration was observed in factor B-deficient mice compared with controls and local reconstitution with granulocytes restored the blistering disease in factor B-deficient mice. Our study provides the first direct evidence for the involvement of the alternative pathway in an autoantibody-induced blistering disease and should facilitate the development of new therapeutic strategies for epidermolysis bullosa acquisita and related autoimmune diseases.<\/jats:p>","DOI":"10.4049\/jimmunol.178.10.6514","type":"journal-article","created":{"date-parts":[[2014,4,18]],"date-time":"2014-04-18T22:53:28Z","timestamp":1397861608000},"page":"6514-6521","source":"Crossref","is-referenced-by-count":81,"title":["The Alternative Pathway of Complement Activation Is Critical for Blister Induction in Experimental Epidermolysis Bullosa Acquisita"],"prefix":"10.1093","volume":"178","author":[{"given":"Sidonia","family":"Mihai","sequence":"first","affiliation":[{"name":"Department of Dermatology, University of Lu\u0308beck , Lu\u0308beck ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mircea T","family":"Chiriac","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of Lu\u0308beck , Lu\u0308beck ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazue","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Laboratory of Developmental Immunology, Massachusetts General Hospital, Harvard Medical School , Boston, MA 02115"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joshua M","family":"Thurman","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Colorado Health Sciences Center , Denver, CO 80218"},{"name":"Department of Immunology, University of Colorado Health Sciences Center , Denver, CO 80218"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V Michael","family":"Holers","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Colorado Health Sciences Center , Denver, CO 80218"},{"name":"Department of Immunology, University of Colorado Health Sciences Center , Denver, CO 80218"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Detlef","family":"Zillikens","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of Lu\u0308beck , Lu\u0308beck ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marina","family":"Botto","sequence":"additional","affiliation":[{"name":"Rheumatology Section, Imperial College School of Medicine , London ,","place":["United Kingdom"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cassian","family":"Sitaru","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of Lu\u0308beck , Lu\u0308beck ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2007,5,15]]},"reference":[{"key":"2025032702492615900_R1","first-page":"861","volume-title":"Exp. 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