Mutations of the Zα domain of ADAR1 result in phenotypes associated with type I interferonopathies suggesting that the disruption of Z-DNA or Z-RNA binding is responsible for disturbed ADAR1 function, resulting in autoimmune disorders like Aicardi-Goutières syndrome (AGS) and Bilateral Striatal Dystonia (BSD) [37,135]⁠. Here, ADAR is linked to Aicardi-Goutières syndrome.