Table 1. Comparison of phenotypes observed in proband and CRISPR/Cas9 Ikbkb mutant mouse.

	Human IKBKBV203I	Mouse IkbkbV203I
CD4+ T cells	Reduced	Reduced
CD8+ T cells	Reduced	Reduced
B cells	Reduced	Reduced
Memory B cells	Reduced	Reduced
T reg cells	Increased or normal	Increased
T cell activation	Increased (naive)	Increased
Phospho-IκBα	Constitutive activation	Constitutive activation