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. 2019 Jun 26;10:1454. doi: 10.3389/fimmu.2019.01454

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Copyright © 2019 Hiroyasu, Turner, Richardson and Granville.

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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Role of proteases in pemphigoid disease. Tissue-type plasminogen activator (tPA) secreted from keratinocytes activates plasminogen to plasmin. Plasmin activates pro-matrix metalloprotease-9 (pro-MMP-9) to MMP-9. MMP-9 degrades α1-antitrypsin (α1-AT). Without inhibition by α1-AT, neutrophil elastase (NE) cleaves hemidesmosome-associated proteins including collagen XVII (COL17) and laminin-332 (lam332). Granzyme B (GzmB) also cleaves hemidesmosome-associating proteins to induce dermal-epidermal separation. GzmB may induce additional neutrophil infiltration through chemoattractant production such as IL-1α, C5a, and COL17/lam332 fragments. ADAM-10 sheds semaphorin-4D (sema4D) to activate autoantibodies (autoAb) production from B cells.