These auto-Abs can be genetically driven, as in patients with autoimmune polyendocrinopathy syndrome type 1 (APS-1) due to AIRE mutations (Bastard et al., 2021d), T cell deficits due to hypomorphic RAG1 or RAG2 mutations (Walter et al., 2015), immune dysregulation, polyendocrinopathy, enteropathy, X-linked due to FOXP3 mutations (Rosenberg et al., 2018), or incontinentia pigmenti due to NEMO mutations (Harris et al., 1992; Bastard et al., 2020). The gene discussed is RAG2; the disease is autoimmune polyendocrine syndrome type 1.