Abstract
The BAFF-receptor (BAFFR) is encoded by the TNFRSF13C gene and is one of the main pro-survival receptors in B cells. Its function is impressively documented in humans by a homozygous deletion within exon 2, which leads to an almost complete block of B cell development at the stage of immature/transitional B cells. The resulting immunodeficiency is characterized by B-lymphopenia, agammaglobulinemia, and impaired humoral immune responses. However, different from mutations affecting pathway components coupled to B cell antigen receptor (BCR) signaling, BAFFR-deficient B cells can still develop into IgA-secreting plasma cells. Therefore, BAFFR deficiency in humans is characterized by very few circulating B cells, very low IgM and IgG serum concentrations but normal or high IgA levels.
Highlights
Reviewed by: Yi Hao, Tongji Medical College, Huazhong University of Science and Technology, China Mike Cancro, University of Pennsylvania, United States
Different from mutations affecting pathway components coupled to B cell antigen receptor (BCR) signaling, BAFFR-deficient B cells can still develop into IgA-secreting plasma cells
The induction of BAFFR expression seems to depend on the expression of functional B cell receptors, it can be maintained in mice after the ablation of SYK, a key element of BCR signaling [19], as well as in cells which were depleted from Ig-α (CD79A), an essential BCR component [20], supporting in both cases the survival of B cells with impaired BCR-signaling
Summary
BAFFR is an atypical representative of the TNF-receptor super-family. Members of this family are typically characterized by several extracellular cysteine-rich domains (CRDs), which serve for ligand binding as well as for ligand-independent assembly of receptor monomers into dimers, trimers or multimers [1,2,3,4,5,6]. After the assembly and cell surface expression of functional IgM molecules, immature IgM+ B cells are tested for their reactivity with self-antigens, which eventually can be corrected by receptor editing. Its expression is upregulated after the expression of functional B cell antigen receptors (BCR) in response to tonic BCR signaling, which enhances BAFFR expression by immature and transitional B cells [15, 18].
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