Mouse RING finger protein 213 (RNF213), which possesses an AAA+ ATPase domain and an E3 ubiquitin ligase domain, is a giant E3 ubiquitin protein ligase consisting of 5,207 amino acids and is associated with autoimmunity, autophagy, angiogenesis and lipid metabolism [13–15]. Here, RNF213 is linked to Autoimmunity.