{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,14]],"date-time":"2026-03-14T06:07:17Z","timestamp":1773468437384,"version":"3.50.1"},"reference-count":51,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T00:00:00Z","timestamp":1773273600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"German Research Foundation","doi-asserted-by":"publisher","award":["EXC 2167\/1, TI-3"],"award-info":[{"award-number":["EXC 2167\/1, TI-3"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"German Research Foundation","doi-asserted-by":"publisher","award":["EM 362\/1-1"],"award-info":[{"award-number":["EM 362\/1-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"name":"CRU 303 Pemphigoid Diseases"},{"name":"CRC 1526 Pathomechanisms of Antibody-mediated Autoimmunity","award":["A01"],"award-info":[{"award-number":["A01"]}]},{"name":"China Scholarship Council and the National Natural Science Foundation of China","award":["81000448"],"award-info":[{"award-number":["81000448"]}]},{"DOI":"10.13039\/501100004168","name":"University of L\u00fcbeck","doi-asserted-by":"publisher","award":["J13-2022"],"award-info":[{"award-number":["J13-2022"]}],"id":[{"id":"10.13039\/501100004168","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004543","name":"China Scholarship Council program","doi-asserted-by":"crossref","award":["202408320122"],"award-info":[{"award-number":["202408320122"]}],"id":[{"id":"10.13039\/501100004543","id-type":"DOI","asserted-by":"crossref"}]},{"name":"RTG2633 Defining and Targeting Autoimmune Pre-Disease"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biomolecules"],"abstract":"<jats:p>Autoantibodies targeting \u03b16\u03b24 integrin have been identified in individual patients with mucous membrane pemphigoid (MMP). Reactivity against \u03b16 integrin has been associated with oral lesions, while anti-\u03b24 integrin reactivity has been linked to ocular involvement. However, the pathogenic effects of these antibodies have not been fully elucidated. Here, we investigated the pathogenic potential of anti-\u03b16 and anti-\u03b24 integrin IgG both in vitro and in vivo. Immune complexes of anti-\u03b16 and anti-\u03b24 integrin induced the release of reactive oxygen species from normal human leukocytes and stimulated CXCL2 secretion in cultured murine C5N keratinocytes. In vivo, repeated injections of IgG against a recombinant fragment of \u03b24 integrin into C57BL\/6 mice led to palpebral conjunctival swelling and mild oral lesions. The latter was observed following injection of IgG against a recombinant fragment of \u03b16 integrin. Histopathological analysis revealed subepithelial inflammatory infiltrates without evidence of split formation. Direct immunofluorescence microscopy showed linear deposits of IgG at the basement membrane zone in most tissues, whereas C3 deposition was largely absent. This lack of complement activation was corroborated by a complement fixation assay, which confirmed that IgG against \u03b16 and \u03b24 integrin failed to induce C3 deposition in normal murine conjunctivae, buccal mucosa, or skin. Collectively, these findings indicate that IgG autoantibodies against \u03b16 and \u03b24 integrin exhibit pathogenic activity in vitro and induce mild disease in vivo, possibly due in part to relatively inefficient complement activation in this model.<\/jats:p>","DOI":"10.3390\/biom16030417","type":"journal-article","created":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T13:12:24Z","timestamp":1773321144000},"page":"417","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Complement Activation May Drive the Pathogenicity of Anti-\u03b16 and Anti-\u03b24 Integrin Antibodies In Vivo"],"prefix":"10.3390","volume":"16","author":[{"given":"Gefei","family":"Du","sequence":"first","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"},{"name":"State Key Laboratory of Oral & Maxillofacial Reconstruction and Regeneration, Key Laboratory of Oral Biomedicine Ministry of Education, Hubei Key Laboratory of Stomatology, School & Hospital of Stomatology, Wuhan University, Wuhan 430079, China"},{"name":"Department of Oral Medicine, School and Hospital of Stomatology, Wuhan University, Wuhan 430079, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7965-8045","authenticated-orcid":false,"given":"Shirin","family":"Emtenani","sequence":"additional","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dennis","family":"Niese","sequence":"additional","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jian","family":"Liu","sequence":"additional","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-3768-1675","authenticated-orcid":false,"given":"Ferdinand","family":"Gebauer","sequence":"additional","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Neele J.","family":"Dunst","sequence":"additional","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aysun","family":"G\u00f6kce","sequence":"additional","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kristina","family":"Spaniol","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University Clinic D\u00fcsseldorf, 40225 D\u00fcsseldorf, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florian","family":"Groeber-Becker","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University Clinic D\u00fcsseldorf, 40225 D\u00fcsseldorf, Germany"},{"name":"Translational Center for Regenerative Therapies (TLC-RT), Fraunhofer Institute for Silicate Research (ISC) W\u00fcrzburg, 97082 Bavaria, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4229-3318","authenticated-orcid":false,"given":"Jelena","family":"\u0160imunovi\u0107","sequence":"additional","affiliation":[{"name":"Genos Glycoscience Research Laboratory, 10000 Zagreb, Croatia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mislav","family":"Novokmet","sequence":"additional","affiliation":[{"name":"Genos Glycoscience Research Laboratory, 10000 Zagreb, Croatia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2034-3687","authenticated-orcid":false,"given":"Gerd","family":"Geerling","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University Clinic D\u00fcsseldorf, 40225 D\u00fcsseldorf, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kyle T.","family":"Amber","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Rush University Medical Center, Chicago, IL 60612, USA"},{"name":"Department of Internal Medicine, Rush University Medical Center, Chicago, IL 60612, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9698-9922","authenticated-orcid":false,"given":"Markus H.","family":"Hoffmann","sequence":"additional","affiliation":[{"name":"Institute for Systemic Inflammation Research, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ralf J.","family":"Ludwig","sequence":"additional","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3855-6683","authenticated-orcid":false,"given":"Katja","family":"Bieber","sequence":"additional","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0355-4290","authenticated-orcid":false,"given":"Stephanie","family":"Goletz","sequence":"additional","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gang","family":"Zhou","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oral & Maxillofacial Reconstruction and Regeneration, Key Laboratory of Oral Biomedicine Ministry of Education, Hubei Key Laboratory of Stomatology, School & Hospital of Stomatology, Wuhan University, Wuhan 430079, China"},{"name":"Department of Oral Medicine, School and Hospital of Stomatology, Wuhan University, Wuhan 430079, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Enno","family":"Schmidt","sequence":"additional","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"},{"name":"Department of Dermatology, Allergology and Venerology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1172-3015","authenticated-orcid":false,"given":"Sabrina","family":"Patzelt","sequence":"additional","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology, University of L\u00fcbeck, 23562 L\u00fcbeck, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,3,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1001\/archderm.138.3.370","article-title":"The First International Consensus On Mucous Membrane Pemphigoid: Definition, Diagnostic Criteria, Pathogenic Factors, Medical Treatment, and Prognostic Indicators","volume":"138","author":"Chan","year":"2002","journal-title":"Arch. 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