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. 2022 Mar 24;13:834342. doi: 10.3389/fimmu.2022.834342

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Copyright © 2022 Koneczny, Tzartos, Mané-Damas, Yilmaz, Huijbers, Lazaridis, Höftberger, Tüzün, Martinez-Martinez, Tzartos and Leypoldt

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Charactristics of IgG4: (A) IgG4 can undergo Fab-arm exchange and form functionally monovalent antibodies. (B) IgG4 may bind and compete for binding of other Ig classes and IgG subclasses. (C) IgG4 has anti-inflammatory properties and lacks typical IgG effector mechanisms. (D) IgG4 autoantibodies can be pathogenic by direct blocking of protein-protein and cell-cell interaction. ADCC, Antibody-dependent cellular cytotoxicity; MuSK, muscle-specific kinase; Lrp4, low density lipoprotein receptor-related protein 4; vWF, von Willebrand factor; ADAMTS13, a disintegrin and metalloprotease with thrombospondin type 1 motif 13.