009 Pathophysiology of desmoglein antibody-negative pemphigus vulgaris

In this study, we showed that he non-desmoglein  (Dsg) AuAbs present in the serum of the Dsg1/3 AuAb-negative acute PV patients are pathogenic, since patients' IgGs induced skin blistering in neonatal mice due to suprabasal acantholysis. The serum level of AuAbs to desmocollin (Dsc)3, M3 muscarinic acetylcholine receptor (M3AR) and the secretory pathway Ca2+/Mn2+-ATPase isoform 1, (SPCA1) encoded by the ATP2C1 gene correlated with the disease stage of PV, and absorption of recombinant Dsc3, M3AR or SPCA1 prevented both skin blistering in the passive transfer of AuAbs model of PV in BALB/c mice and significantly decreased extent of acanthol ysis in the neonatal mouse skin explant model.

Source: Journal of Investigative Dermatology - April 19, 2019 Category: Dermatology Authors: K. Amber, A. Chernyavsky, A. Agnoletti, C. Wang, S.A. Grando Tags: Adaptive and Auto-Immunity Source Type: research