Staniek Julian, Rizzi Marta
Department of Rheumatology and Clinical Immunology, Faculty of Medicine, University Medical Center Freiburg, University of Freiburg, Freiburg, Germany.
Faculty of Medicine, Center for Chronic Immunodeficiency, University Medical Center Freiburg, University of Freiburg, Freiburg, Germany.
Immunol Rev. 2025 Mar;330(1):e70004. doi: 10.1111/imr.70004.
The differentiation of naive follicular B cells into either the germinal center (GC) or extrafollicular (EF) pathway plays a critical role in shaping the type, affinity, and longevity of effector B cells. This choice also governs the selection and survival of autoreactive B cells, influencing their potential to enter the memory compartment. During the first 2-3 days following antigen encounter, initially activated B cells integrate activating signals from T cells, Toll-like receptors (TLRs), and cytokines, alongside inhibitory signals mediated by inhibitory receptors. This integration modulates the intensity of signaling, particularly of the PI3K/AKT/mTOR pathway, which plays a central role in guiding developmental decisions. These early signaling events determine whether B cells undergo GC maturation or differentiate rapidly into antibody-secreting cells (ASCs) via the EF pathway. Dysregulation of these signaling pathways-whether through excessive activation or defective regulatory mechanisms-can disrupt the balance between GC and EF fates, predisposing individuals to autoimmunity. Accordingly, aberrant PI3K/AKT/mTOR signaling has been implicated in the defective selection of autoreactive B cells, increasing the risk of autoimmune disease. This review focuses on the signaling events in newly activated B cells, with an emphasis on the induction and regulation of the PI3K/AKT/mTOR pathway. It also highlights gaps in our understanding of how alternative B cell fates are regulated. Both the physiological context and the implications of inborn errors of immunity (IEIs) and complex autoimmune conditions will be discussed in this regard.
初始滤泡B细胞向生发中心(GC)或滤泡外(EF)途径的分化在塑造效应B细胞的类型、亲和力和寿命方面起着关键作用。这种选择还决定了自身反应性B细胞的选择和存活,影响它们进入记忆库的潜力。在遇到抗原后的最初2 - 3天内,最初活化的B细胞整合来自T细胞、Toll样受体(TLR)和细胞因子的激活信号,以及由抑制性受体介导的抑制信号。这种整合调节信号传导的强度,特别是PI3K/AKT/mTOR途径的信号传导强度,该途径在指导发育决策中起核心作用。这些早期信号事件决定B细胞是经历GC成熟还是通过EF途径迅速分化为抗体分泌细胞(ASC)。这些信号通路的失调——无论是通过过度激活还是有缺陷的调节机制——都可能破坏GC和EF命运之间的平衡,使个体易患自身免疫性疾病。因此,异常的PI3K/AKT/mTOR信号传导与自身反应性B细胞的缺陷选择有关,增加了自身免疫性疾病的风险。本综述重点关注新活化B细胞中的信号事件,重点是PI3K/AKT/mTOR途径的诱导和调节。它还强调了我们在理解替代B细胞命运如何被调节方面的差距。在这方面将讨论免疫缺陷先天性疾病(IEI)和复杂自身免疫性疾病的生理背景及影响。