For instance, Aicardi-Goutières syndrome mutations in RNase H2 (cleaves RNA-DNA hybrids) or in adenosine deaminase acting on RNA 1 (ADAR1) invoke innate immune sensing of self-nucleic acids (DNA or RNA), leading to type I IFN signaling and systemic inflammation (13). Here, ADAR is linked to Aicardi-Goutieres syndrome.