In one case of AGS, there was a simultaneous mutation of the amino acid sites Asn173 to Ser and Pro193 to Ala in the ZαADAR1, both of which are key amino acid sites for ZαADAR1 binding to Z-NA, and mutation of these two sites severely disrupted the binding of ADAR1 to Z-NA (83). The gene discussed is ADAR; the disease is Aicardi-Goutieres syndrome.