In contrast to the accumulation of ubiquitin-coupled proteins in the cells of patients with PRAAS (12), the total spleen cells of Psmb8-KI mice did not show increased accumulation of ubiquitin, although the patterns of chymotrypsin, trypsin, and caspase activities were distinct between control and Psmb8-KI mice. Here, PSMB8 is linked to proteosome-associated autoinflammatory syndrome.