As RIG-I and MDA5 are associated with autoimmune diseases including Aicardi-Goutières syndrome (AGS, a severe autoimmune encephalopathy caused by aberrant activation of the IFN-I axis), Singleton–Merten syndrome(SMS, a type I interferonopathy characterized by aortic calcifications, psoriasis, glaucoma and skeletal abnormalities) and type 1 diabetes (T1D), TRIM40 may attenuate the pathogenesis of autoimmune diseases by regulating the activation of RIG-I and MDA5 (59–61). Here, RIGI is linked to Aicardi-Goutières syndrome.