By diminishing the interactions of RNase H2B with ZMYM3, it is conceivable that the AGS-mutations investigated here may reduce the stability of the RNase H2 complex, as has been observed for several other AGS-associated RNase H2 mutations that compromise the interaction between the C and A or C and B subunits [13,30]. Here, ZMYM3 is linked to Aicardi-Goutieres syndrome.