Elucidation of the pathogenesis of bullous pemphigoid by molecular imaging of collagen XVII

本研究揭示了大疱性类天疱疮(BP)患者的免疫球蛋白自身抗体直接与细胞表面的XVII胶原(COL17)结合，导致复合体动态内化到细胞内。此外，COL17脱落胞外区上的新表位被发现是来自BP患者的某些自身抗体的靶标。此外，本研究还揭示了迁移中的角质形成细胞直接释放COL17，COL17与包括层粘连蛋白332和IV型胶原在内的其他细胞外基质蛋白结合。

This study revealed that IgG autoantibodies from bullous pemphigoid (BP) patients directly bind with collagen XVII (COL17) on the cell-surface, resulting in dynamic internalization of the complex into the cells. Furthermore, neoepitopes on the shed ectodomain of COL17 were found to be targeted by certain autoantibodies from BP patients. In addition, this study disclosed that migrating keratinocytes directly shed the COL17 which binds with other extracellular matrix proteins including laminin332 and collagen IV.