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

From: Biological characteristics of transcription factor RelB in different immune cell types: implications for the treatment of multiple sclerosis

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 [48,49,50,51,52,53,54]
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]
  1. 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