Fig. 6

Activation of pyrin inflammasome in patients with pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome. Mutations of PSTPIP1 in patients with PAPA (e.g., A230T and E250Q) result in hyperphosphorylation of PSTPIP1, which strengthens its interaction with pyrin via the B-box domain to activate the pyrin inflammasome