Several autoimmune diseases, including the Aicardi-Goutières and Singleton-Merten syndromes (SMS), were linked to single amino acid mutations in the SF2 domains of MDA5 and RIG-I (Funabiki et al., 2014; Jang et al., 2015; Rice et al., 2014; Rutsch et al., 2015). Here, RIGI is linked to Smith-Magenis syndrome.