IFNA1 and Aicardi-Goutieres syndrome: In AGS, the primary source of IFN-α is the CNS, and the microangiopathy and brain disease is strongly associated with CNS-specific IFN-α (Cuadrado et al., 2013; Klok et al., 2015; Lebon et al., 1983; Lodi et al., 2021; van Heteren et al., 2008; Viengkhou et al., 2024b).