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BP180 is currently considered as the major pathogenic autoantigen. However, previous clinical findings suggested that anti\u2010BP230 autoantibodies alone can cause skin lesions in animal models and many BP patients. The characteristics of BP230 and the pathogenic roles of anti\u2010BP230 antibodies have been proposed. First, at the molecular level, BP230 mediates the attachment of keratin intermediate filaments to the hemidesmosomal plaque and interacts with other constituents of hemidesmosomes. Second, the presence of BP230 autoantibodies may correlate with specific clinical features of BP. The immunoglobulin (Ig)G autoantibodies from BP patients react mainly against the C\u2010terminus of BP230, while the IgE autoantibodies are still inconclusive. Third, <jats:italic>in vivo<\/jats:italic>, autoantibodies against BP230 involved in the disease may not only induce the inflammatory response but also impair the structural stability of hemidesmosomes. This article reviews recently published work about the role of BP230 and its antibodies, including IgG and IgE, aiming to find clues of its clinical association and lay the foundation for the research on the pathogenicity of antibodies against BP230.<\/jats:p>","DOI":"10.1111\/1346-8138.15251","type":"journal-article","created":{"date-parts":[[2020,2,12]],"date-time":"2020-02-12T07:23:41Z","timestamp":1581492221000},"page":"317-326","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Role of BP230 autoantibodies in bullous pemphigoid"],"prefix":"10.1111","volume":"47","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5424-1331","authenticated-orcid":false,"given":"Yen-chi","family":"Shih","sequence":"first","affiliation":[{"name":"Department of Dermatology School of Medicine Ruijin Hospital Shanghai Jiao Tong University Shanghai 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