A notable example is the Aicardi-Goutières syndrome (AGS), a leukodystrophy caused by TREX1 mutations that result in chronic IFN-I production via sustained cGAS activation, leading to neuroinflammation and immune-mediated demyelination (Giroud et al., 1986; Crow and Manel, 2015). Here, TREX1 is linked to Aicardi-Goutieres syndrome.