Patients with partial recombination-activating gene (RAG) deficiency (pRD) present variable late-onset autoimmune clinical phenotypes. Walter and colleagues identified a restricted primary B cell antigen receptor repertoire enriched for autoreactivity and clonal persistence in pRD. They described dysregulated B cell maturation with expansion of T-bet+ B cells revealing how RAG impacts stringency of tolerance and B cell fate in the periphery.
- Krisztian Csomos
- Boglarka Ujhazi
- Jolan E. Walter
