Table 1 RelB deficiency can lead to a range of immune disorders in both mice and humans

Thymus

highly disorganized medullary architecture; absent of mTECs and DCs, particularly Aire+ mTECs; impaired negative selection in T cells;

thymic dysplasia; lack of Hassall’s corpuscles;

LTβR CD40 RANK

[48,49,50,51,52,53,54]

SLOs

lack of Peyer’s patches and peripheric lymph nodes; splenic structural damage: impaired FDCs network; dispersed reticular fibroblast network throughout the white pulp; deficient GC and marginal zone development; decreased BCL and SCL production;

/

LTβR

[53, 55,56,57]

DCs

decreased surface markers: MHC class-II, CD11c, CD80, CD86 and CD40; lower capacity of antigen presentation and T cell activation;

/

AhR

[24, 52, 58,59,60,61]

T cells

reduced IFN-γ; damaged T cell differentiation and T cell immunity; multiorgan inflammation; significantly elevated migratory activity of effector memory T cell;

T cell dysmaturity; decreased T cell output from thymus; abnormal T cell subtypes clonal expansion; significantly reduced IFN-γ and IL-2 generation; decreased expression of T-bet and STAT1; evere T cell immunodeficiency;

/

[48, 53, 62,63,64]

B cells

reduced follicular B cells; Absent marginal zone B cells; B-cell progenitors developmental disorders; remarkable reduction of peripheral mature B cells;

barricaded B cell development; shortage of specific antibodies; severe B cell immunodeficiency; lack of CD27+ memory B cells; decreased expression of BAFFR; impaired CD40 signaling;

BAFFR CD40

[48, 56, 65,66,67]