The lack of proper ATP hydrolysis by RNA sensors, such as RIG-I and MDA5, was recently linked to many genetic disorders whose pathogenesis is caused by an upregulated type 1 IFN signaling that leads to many autoimmune disorders such as Aicardi-Goutières syndrome (AGS), Singleton-Merten syndrome (SMS), systemic lupus erythematosus (SLE), and type 1 diabetes [46]. The gene discussed is RIGI; the disease is Aicardi-Goutieres syndrome.