Analysis of serum autoantibodies from 56 AGS patients (23.4%-TREX1; 57.1%-RNASEH2B; 2.1% RNASEH2A; 4.3%; 8.5% RNASEH2C; 4.3% SAMHD1; and 4.3% ADAR1 mutants) was performed, using an autoantibody array to assess their antigen-specificity. Here, SAMHD1 is linked to Aicardi-Goutieres syndrome.