Article ; Online: BAFF receptor polymorphisms and deficiency in humans.
2021 Volume 71, Page(s) 103–110
Abstract: The BAFF-receptor (BAFFR) is a member of the TNF-receptor family. It is expressed only by B cells and binds BAFF as single ligand, which activates key signaling pathways regulating essential cellular functions, including survival, protein synthesis, and ... ...
| Abstract | The BAFF-receptor (BAFFR) is a member of the TNF-receptor family. It is expressed only by B cells and binds BAFF as single ligand, which activates key signaling pathways regulating essential cellular functions, including survival, protein synthesis, and metabolic fitness. In humans, BAFFR deficiency interrupts B cell development at the transition from immature to mature B cells and causes B lymphopenia, hypogammaglobulinemia, and impaired humoral immune responses. Polymorphisms in TNFRSF13C gene affecting BAFFR oligomerization and signaling have been described in patients with immunodeficiency, autoimmunity and B cell lymphomas. Despite a uniform expression pattern of BAFFR in peripheral mature B cells, depletion of BAFF with neutralizing antibodies in patients with systemic lupus erythematosus does not affect the survival of switched memory B cells. These findings imply a distinct dependency of mature B cell subsets on BAFF/BAFFR interaction and highlight the contribution of BAFFR-derived signals in peripheral B cell development and homeostasis. |
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| MeSH term(s) | B-Cell Activating Factor/immunology ; B-Cell Activation Factor Receptor/deficiency ; B-Cell Activation Factor Receptor/genetics ; B-Cell Activation Factor Receptor/immunology ; B-Lymphocytes/immunology ; Homeostasis/immunology ; Humans ; Polymorphism, Genetic/genetics |
| Chemical Substances | B-Cell Activating Factor ; B-Cell Activation Factor Receptor ; TNFRSF13C protein, human ; TNFSF13B protein, human |
| Language | English |
| Publishing date | 2021-07-24 |
| Publishing country | England |
| Document type | Journal Article ; Research Support, Non-U.S. Gov't ; Review |
| ZDB-ID | 1035767-1 |
| ISSN | 1879-0372 ; 0952-7915 |
| ISSN (online) | 1879-0372 |
| ISSN | 0952-7915 |
| DOI | 10.1016/j.coi.2021.06.008 |
| Database | MEDical Literature Analysis and Retrieval System OnLINE |
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