Almaden Jonathan V, Liu Yi C, Yang Edward, Otero Dennis C, Birnbaum Harry, Davis-Turak Jeremy, Asagiri Masataka, David Michael, Goldrath Ananda W, Hoffmann Alexander
Signaling Systems Laboratory and San Diego Center for Systems Biology, University of California, San Diego, La Jolla, CA;
Signaling Systems Laboratory and San Diego Center for Systems Biology, University of California, San Diego, La Jolla, CA; Department of Microbiology, Immunology, and Molecular Genetics, University of California, Los Angeles, Los Angeles, CA;
Blood. 2016 Mar 10;127(10):1276-86. doi: 10.1182/blood-2014-10-606988. Epub 2016 Jan 14.
Targeted deletion of BAFF causes severe deficiency of splenic B cells. BAFF-R is commonly thought to signal to nuclear factor κ-light-chain-enhancer of activated B cells (NF-κB)-inducing kinase dependent noncanonical NF-κB RelB. However, RelB-deficient mice have normal B-cell numbers. Recent studies showed that BAFF also signals to the canonical NF-κB pathway, and we found that both RelB and cRel are persistently activated, suggesting BAFF signaling coordinates both pathways to ensure robust B-cell development. Indeed, we report now that combined loss of these 2 NF-κB family members leads to impaired BAFF-mediated survival and development in vitro. Although single deletion of RelB and cRel was dispensable for normal B-cell development, double knockout mice displayed an early B-cell developmental blockade and decreased mature B cells. Despite disorganized splenic architecture in Relb(-/-)cRel(-/-) mice, generation of mixed-mouse chimeras established the developmental phenotype to be B-cell intrinsic. Together, our results indicate that BAFF signals coordinate both RelB and cRel activities to ensure survival during peripheral B-cell maturation.
BAFF的靶向缺失导致脾脏B细胞严重缺乏。通常认为BAFF-R向活化B细胞核因子κ轻链增强子(NF-κB)诱导激酶依赖性非经典NF-κB RelB发出信号。然而,RelB缺陷小鼠的B细胞数量正常。最近的研究表明,BAFF也向经典NF-κB途径发出信号,并且我们发现RelB和cRel均持续活化,这表明BAFF信号传导协调这两条途径以确保强大的B细胞发育。实际上,我们现在报告这两个NF-κB家族成员的联合缺失导致体外BAFF介导的存活和发育受损。尽管RelB和cRel的单基因缺失对于正常B细胞发育是可有可无的,但双敲除小鼠表现出早期B细胞发育阻滞且成熟B细胞减少。尽管Relb(-/-)cRel(-/-)小鼠的脾脏结构紊乱,但混合小鼠嵌合体的产生确定了发育表型是B细胞内在的。总之,我们的结果表明BAFF信号传导协调RelB和cRel的活性,以确保外周B细胞成熟过程中的存活。