062 Granzyme B-mediated cleavage of hemidesmosomal proteins contributes to blister formation in epidermolysis bullosa acquisita

Granzyme B (GzmB) is a serine protease that exhibits both intracellular and extracellular roles in immune cell-mediated apoptosis and extracellular proteolysis, respectively. GzmB-positive neutrophils are abundant in areas proximal to blisters in autoimmune subepidermal blistering diseases and may contribute to pathology. In the current study, the function of GzmB in blistering was assessed in an anti-collagen VII IgG passive transfer murine model of epidermolysis bullosa acquisita (EBA) using both wild type C57Bl/6 (WT) and GzmB knockout (GzmBKO) mice.

Source: Journal of Investigative Dermatology - April 19, 2019 Category: Dermatology Authors: S. Hiroyasu, M.R. Zeglinski, H. Zhao, P.A. Lennox, N. Van Laeken, N.J. Carr, A. Kasprick, R.J. Ludwig, D.J. Granville Tags: Adaptive and Auto-Immunity Source Type: research