Mutations of seven genes, including RNaseH2A (AGS4), RNaseH2B (AGS2), RNaseH2C (AGS3), and ADAR1 (AGS6), have been identified in association with AGS, and ten AGS6 mutations of ADAR1 have been reported so far19. The gene discussed is RNASEH2A; the disease is Aicardi-Goutieres syndrome.