Table 1.
RelB deficiency can lead to a range of immune disorders in both mice and humans
| Tissue or cell types | Immune disorders in mice with RelB deficiency | Immune disorders in humans with RelB deficiency | Related signaling | References |
|---|---|---|---|---|
| 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 | [48–54] |
| 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, 55–57] |
| DCs | decreased surface markers: MHC class-II, CD11c, CD80, CD86 and CD40; lower capacity of antigen presentation and T cell activation; | / | AhR | [24, 52, 58–61] |
| 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, 62–64] |
| 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, 65–67] |
Abbreviations: mTECs medullary thymic epithelial cells; DCs dendritic cells; Aire autoimmune regulator; SLOs secondary lymphoid organs; FDCs follicular dendritic cells; GC germinal center; nTregs natural regulatory T cells; SLC secondary lymphoid tissue chemokine; BLC B lymphocyte chemoattractant; FoxP3 Forkhead box protein 3; AhR aryl hydrocarbon receptor; IFN-γ interferon-γ; STAT1 signal transducer and activator of transcription 1; RANK receptor activator of NF-κB; LTβR lymphotoxin β receptor; BAFFR B cell activating factor receptor