CD14 and Aicardi-Goutieres syndrome: IFN type 1 gene expression was assessed using a set of ISG first used in studies of Aicardi-Goutières Syndrome, a prototypical monogenic type 1 interferonpathy.26 These data demonstrated reduced expression of mitochondrial genes was associated with increased expression of ISG in JDM pretreatment CD14+monocytes (figure 1E).