Department of Microbiology & Immunology, Penn State College of Medicine, Hershey, Pennsylvania, United States of America.
Department of Pathology, Penn State College of Medicine, Hershey, Pennsylvania, United States of America.
PLoS One. 2021 Apr 23;16(4):e0250340. doi: 10.1371/journal.pone.0250340. eCollection 2021.
Experimental autoimmune encephalomyelitis (EAE) is an established animal model of multiple sclerosis (MS). Inflammatory CD4+ T cell responses directed against CNS antigens, including myelin proteolipid protein (PLP), are key mediators of EAE. Dendritic cells (DCs) are critical for the induction of T cell responses against infectious agents. However, the importance of DCs in priming self-reactive CD4+ T cells in autoimmune disease such as MS has been unclear. To determine the requirement of DCs in PLP-specific CD4+ T cell responses and EAE, we genetically deleted CD11c+ DCs in PLP T cell receptor (TCR) transgenic SJL mice constitutively. DC deficiency did not impair the development, selection or the pathogenic function of PLP-specific CD4+ T cells in these mice, and resulted in accelerated spontaneous EAE compared to DC sufficient controls. In addition, using a genetic approach to ablate DCs conditionally in SJL mice, we show that CD11c+ DCs were dispensable for presenting exogenous or endogenous myelin antigen to PLP-specific T cells and for promoting pro-inflammatory T cell responses and severe EAE. Our findings demonstrate that constitutive or conditional ablation of CD11c+ DCs diminished self-tolerance to PLP autoantigen. They further show that in the absence of DCs, non-DCs can efficiently present CNS myelin antigens such as PLP to self-reactive T cells, resulting in accelerated onset of spontaneous or induced EAE.
实验性自身免疫性脑脊髓炎 (EAE) 是多发性硬化症 (MS) 的一种成熟的动物模型。针对中枢神经系统抗原(包括髓鞘蛋白脂蛋白)的炎症性 CD4+T 细胞反应是 EAE 的关键介质。树突状细胞 (DC) 对于针对感染因子的 T 细胞反应的诱导至关重要。然而,DC 在自身免疫性疾病(如 MS)中引发自身反应性 CD4+T 细胞中的重要性尚不清楚。为了确定 DC 在 PLP 特异性 CD4+T 细胞反应和 EAE 中的必要性,我们在 SJL 小鼠的 PLP T 细胞受体 (TCR) 转基因中基因缺失了 CD11c+DCs 。DC 缺失不会损害这些小鼠中 PLP 特异性 CD4+T 细胞的发育、选择或致病性功能,并且与 DC 充足的对照相比,导致自发性 EAE 加速。此外,我们使用一种遗传方法在 SJL 小鼠中条件性地缺失 DCs,表明 CD11c+DCs 对于向 PLP 特异性 T 细胞呈递外源性或内源性髓鞘抗原以及促进促炎 T 细胞反应和严重的 EAE 是可有可无的。我们的发现表明,CD11c+DCs 的组成性或条件性缺失会降低对 PLP 自身抗原的自身耐受性。它们进一步表明,在没有 DC 的情况下,非 DC 可以有效地将中枢神经系统髓鞘抗原(如 PLP)呈递给自身反应性 T 细胞,导致自发性或诱导性 EAE 的发作加速。