SAMHD1 was originally described as a factor whose mutations are associated with an autoimmune conditions designated Aicardi–Goutières syndrome (AGS) with clinical manifestations resembling congenital viral infection and characterized by a high expression of type I interferon (IFN) and upregulation of IFN-stimulated genes [29]. Here, SAMHD1 is linked to Aicardi-Goutieres syndrome.