While initial reports of SAMHD1 associated RNase activity were strongly questioned by the community because of the reversible nature of SAMHD1 restriction and due to potential co-purification of unknown RNase(s) (37, 47, –, 49), recent evidence links SAMHD1 RNase activity to the inflammatory phenotype in Aicardi-Goutières syndrome (50). The gene discussed is SAMHD1; the disease is Aicardi-Goutières syndrome.