SKIC2 and trichohepatoenteric syndrome: For example, ADAR1 (adenosine deaminase acting on RNA 1) mutations are associated with both Aicardi-Goutières syndrome (AGS) and T1D; SKIV2L mutations are associated with both SLE and trichohepatoenteric syndrome; AGS-associated SAMHD1 mutants are significantly upregulated and correlated with autoinflammation in SLE patients.30 In addition, disruptions of RBP expression cause diversified phenotypes of chronic inflammation and AIDs in mice, in a manner dependent on the cell type or developmental stages upon conditional gene ablation (Table 2).