Jocelyn R. Farmer

The paper identifies loss of transitional B cell tolerance and extrafollicular expansion of autoreactive B cells as a common mechanism driving autoimmunity in monogenic CVID.

Congenital T cell activation in CTLA4-deficient patients impairs transitional to follicular B cell maturation and can be rescued by CTLA4 replacement therapy in vivo.

This study shows that NF-κB signaling is necessary for maintaining X-chromosome inactivation in female T cells after activation.