PNPT1 and Aicardi-Goutieres syndrome: Certainly in the autoimmune vasculitis variants known as Aicardi-Goutières syndrome, where altered degradation of cytosolic DNA or RNA is caused by mutations in TREX1/SAMHD1/RNASEH2A/B/C/ADAR/IFIH1/PNPT1, the progressive immune activation leads to phenotypes of neuro-inflammation/-degeneration [66, 67].