Pokatayev et al. (2016) introduced an RNase H2 AGS mutation into the mouse RNASEH2A gene, resulting in homozygous RNASEH2A-G37S/G37S (G37S) mice. The analogous mutation causes severe early onset AGS in humans. The mouse mutant had reduced RNase activity and increased levels of cytosolic L1 DNA (Pokatayev et al., 2016). The gene discussed is RNASEH2A; the disease is Aicardi-Goutieres syndrome.