Transgenic Rescue of Desmoglein 3 Null Mice with Desmoglein 1 to Develop a Syngeneic Mouse Model for Pemphigus Vulgaris

The study developed a syngeneic mouse model for pemphigus vulgaris (PV) by using transgenic expression of desmoglein 1 (Dsg1) to rescue B6-Dsg3−/− mice, which typically did not survive due to severe oral erosions. By expressing Dsg1, the researchers were able to block the pathogenic effects of anti-Dsg3 antibodies, improving survival rates and reducing symptoms such as body weight loss and telogen hair loss. Over 80% of the B6-Dsg1tg/tgDsg3−/− mice survived at week 6, compared to the B6-Dsg1wt/wtDsg3−/− mice, which died by week 2. This model provided a valuable tool for studying the immunological mechanisms in autoimmunity and tolerance of Dsg3.