{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T02:23:21Z","timestamp":1789611801163,"version":"build-2803163510"},"reference-count":55,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2012,5,8]],"date-time":"2012-05-08T00:00:00Z","timestamp":1336435200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["pathsocjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Pathology"],"published-print":{"date-parts":[[2012,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Autoantibody\u2010mediated diseases are clinically heterogeneous and often fail conventional therapeutic strategies. Gene expression profiling has helped to identify new molecular pathways in these diseases, although their potential as treatment targets largely remains to be functionally validated. Based on weighted gene co\u2010expression network analysis, we determined the transcriptional network in experimental epidermolysis bullosa acquisita (EBA), a paradigm of an antibody\u2010mediated organ\u2010specific autoimmune disease characterized by autoantibodies directed against type VII collagen. We identified 33 distinct and differentially expressed modules, including Fc\u03b3 receptor (Fc\u03b3R) IV and components of the neutrophil\u2010associated enzyme system in autoantibody transfer\u2010induced EBA. Validation experiments, including functional analysis, demonstrated that Fc\u03b3RIV expression on neutrophils crucially contributes to autoantibody\u2010induced tissue injury in the transfer model of EBA. Mice lacking the common \u03b3\u2010chain of activating Fc\u03b3Rs, deficient in Fc\u03b3RIV or treated with Fc\u03b3RIV function blocking antibody, but not mice deficient in Fc\u03b3RI, Fc\u03b3RIIB, Fc\u03b3RIII or both Fc\u03b3RI and Fc\u03b3RIII, were effectively protected from EBA. Skin disease was restored in \u03b3\u2010chain\u2010deficient mice locally reconstituted with neutrophils from wild\u2010type, but not from \u03b3\u2010chain\u2010deficient, mice. Our findings both genetically and functionally identify a novel disease\u2010related molecule, Fc\u03b3RIV, in an autoantibody\u2010mediated disorder, which may be of importance for the development of novel targeted therapies. Copyright \u00a9 2012 Pathological Society of Great Britain and Ireland. Published by John Wiley &amp; Sons, Ltd.<\/jats:p>","DOI":"10.1002\/path.4023","type":"journal-article","created":{"date-parts":[[2012,3,19]],"date-time":"2012-03-19T05:29:49Z","timestamp":1332134989000},"page":"8-19","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":86,"title":["Genetic identification and functional validation of Fc\u03b3RIV as key molecule in autoantibody\u2010induced tissue 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