006 Pathogenic autoantibody derived from Treg-deficient scurfy mice targets Type VII Collagen and induces Epidermolysis bullosa acquisita-like blistering disease

Abstract

Missing regulatory T cell (Treg) control contributes to the development of different autoimmune diseases. Scurfy mice have a missense mutation in the transcription factor Foxp3 which leads to dysfunctional Tregs. Previously, we have shown that scurfy mice develop high titers of autoantibodies with reactivity to structural proteins in the skin. Furthermore, the development of a pathogenic autoantibody that targets BP230 and induces a bullous pemphigoid-like phenotype indicates the progression of autoimmune blistering diseases (AIBD) in the absence of functional Tregs.