SAMHD1 and Aicardi-Goutieres syndrome: Mutations in SAMHD1 are associated with a rare genetic disorder known as Aicardi-Goutieres syndrome (AGS) with symptoms of a congenital viral infection likely due to excessive production of IFN-alpha (IFNα) and increased immune activation[22,23], suggesting that SAMHD1 may act as a negative regulator of the type I IFN response.