The RNase H2 complex is composed of a catalytic A subunit (encoded by Rnaseh2a, 69724) and a second scaffolding B subunit (Rnaseh2b, 67153) in addition to subunit C. In humans, mutations in any of these three genes result in an autosomal recessive neurological autoinflammatory disorder called Aicardi-Goutières Syndrome (AGS) [13]. This evidence concerns the gene RNASEH2A and Aicardi-Goutieres syndrome.