Nakajo–Nishimura syndrome (NNS), also known as proteasome-associated autoinflammatory syndrome 1 (PRAAS1, OMIM; 256040) is a disease characterized by chronic inflammation and lipomuscular atrophy caused by homozygous loss-of-function mutations in the PSMB8 gene encoding β5i, a component of the immunoproteasome [46,47]. Here, PSMB8 is linked to proteosome-associated autoinflammatory syndrome.