Zhu Xingxing, Wu Yue, Li Yanfeng, Zhou Xian, Watzlawik Jens O, Chen Yin Maggie, Raybuck Ariel L, Billadeau Daniel, Shapiro Virginia, Springer Wolfdieter, Sun Jie, Boothby Mark R, Zeng Hu
Division of Rheumatology, Department of Medicine, Mayo Clinic Rochester, MN 55905, USA.
Carter Immunology Center, University of Virginia, Charlottesville, VA 22908, USA.
bioRxiv. 2024 Mar 12:2024.02.26.582122. doi: 10.1101/2024.02.26.582122.
During the humoral immune response, B cells undergo rapid metabolic reprogramming with a high demand for nutrients, which are vital to sustain the formation of the germinal centers (GCs). Rag-GTPases sense amino acid availability to modulate the mechanistic target of rapamycin complex 1 (mTORC1) pathway and suppress transcription factor EB (TFEB) and transcription factor enhancer 3 (TFE3), members of the microphthalmia (MiT/TFE) family of HLH-leucine zipper transcription factors. However, how Rag-GTPases coordinate amino acid sensing, mTORC1 activation, and TFEB/TFE3 activity in humoral immunity remains undefined. Here, we show that B cell-intrinsic Rag-GTPases are critical for the development and activation of B cells. RagA/RagB deficient B cells fail to form GCs, produce antibodies, and generate plasmablasts in both T-dependent (TD) and T-independent (TI) humoral immune responses. Deletion of RagA/RagB in GC B cells leads to abnormal dark zone (DZ) to light zone (LZ) ratio and reduced affinity maturation. Mechanistically, the Rag-GTPase complex constrains TFEB/TFE3 activity to prevent mitophagy dysregulation and maintain mitochondrial fitness in B cells, which are independent of canonical mTORC1 activation. TFEB/TFE3 deletion restores B cell development, GC formation in Peyer's patches and TI humoral immunity, but not TD humoral immunity in the absence of Rag-GTPases. Collectively, our data establish Rag-GTPase-TFEB/TFE3 pathway as an mTORC1 independent mechanism to coordinating nutrient sensing and mitochondrial metabolism in B cells.
在体液免疫反应过程中,B细胞经历快速的代谢重编程,对营养物质有很高的需求,这些营养物质对于维持生发中心(GC)的形成至关重要。Rag-GTP酶感知氨基酸可用性,以调节雷帕霉素复合物1(mTORC1)途径,并抑制转录因子EB(TFEB)和转录因子增强子3(TFE3),它们是HLH-亮氨酸拉链转录因子的小眼畸形(MiT/TFE)家族成员。然而,Rag-GTP酶在体液免疫中如何协调氨基酸感知、mTORC1激活和TFEB/TFE3活性仍不清楚。在这里,我们表明B细胞内在的Rag-GTP酶对B细胞的发育和激活至关重要。RagA/RagB缺陷的B细胞在T细胞依赖(TD)和T细胞非依赖(TI)的体液免疫反应中均无法形成GC、产生抗体和生成浆母细胞。在GC B细胞中删除RagA/RagB会导致暗区(DZ)与亮区(LZ)比例异常,并降低亲和力成熟。从机制上讲,Rag-GTP酶复合物限制TFEB/TFE3活性,以防止B细胞中的线粒体自噬失调并维持线粒体健康,这独立于经典的mTORC1激活。在没有Rag-GTP酶的情况下,TFEB/TFE3缺失可恢复B细胞发育、派尔集合淋巴结中的GC形成和TI体液免疫,但不能恢复TD体液免疫。总的来说,我们的数据确立了Rag-GTP酶-TFEB/TFE3途径是一种独立于mTORC1的机制,用于协调B细胞中的营养感知和线粒体代谢。