Type XVII collagen (COL17) and the non-collagenous 16A (NC16A) domain is regarded as the major pathogenic domains for bullous pemphigoid (BP). Some patients with BP have autoantibodies against parts of COL17 outside the NC16A domain (hereinafter the non-NC16A domain) and show less inflammatory manifestations. There were no significant differences in titres and IgG subclasses between NC16A-BP and non-NC16A-BP as determined by indirect immunofluorescent microscopy. The neutrophil activation capacities determined by ROS release did not differ between NC16A-BP and non-NC16A-BP. However, NC16A-BP IgG depleted COL17 in a dose-dependent manner. Treatment with NC16A-BP IgG, but not with non-NC16A-BP IgG, significantly decreased the adhesion strength. We speculate that the differences in clinical severity between NC16A-BP and non-NC16A-BP relate to the degree of COL17 depletion.
Keywords: bullous pemphigoid; depletion; internalization; non-collagenous 16A domain; type XVII collagen.
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