These diseases include proteasome-associated autoinflammatory syndromes (or PRAAS), caused by additive loss-of-function mutations in genes related to proteasome components (such as PSMB8, PSMB9, PSMB7, PSMA3 or proteasome assembly factors as POMP and PSMG2), and STING-associated vasculopathy with onset during infancy (or SAVI), caused by gain-of-function mutations in the TMEM173 gene, which encodes the stimulator of IFN genes (STING) protein, an evolutionarily conserved endoplasmic reticulum transmembrane protein with the downstream effect of activating IFN pathway [60]. The gene discussed is STING1; the disease is proteosome-associated autoinflammatory syndrome.