BAFF and BAFF-Receptor in B Cell Selection and Survival
Open Access
- 8 October 2018
- journal article
- review article
- Published by Frontiers Media SA in Frontiers in Immunology
- Vol. 9, 2285
- https://doi.org/10.3389/fimmu.2018.02285
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.Keywords
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