Department of Medicine, Translational Transplant Research Center, Precision Institute of Immunology, Icahn School of Medicine at Mount Sinai, New York, New York, USA.
Nephrology Dialysis and Renal Transplantation Unit, S. Orsola University Hospital, Bologna, Italy.
JCI Insight. 2019 Apr 23;5(10):127428. doi: 10.1172/jci.insight.127428.
IL-17-producing CD4+ cells (TH17) are pathogenically linked to autoimmunity including to autoimmune kidney disease. Erythropoietin's (EPO) newly recognized immunoregulatory functions and its predominant intra-renal source suggested that EPO physiologically regulates TH17 differentiation, thereby serving as a barrier to the development of autoimmune kidney disease. Using in vitro studies of human and murine cells and in vivo models, we show that EPO ligation of its receptor (EPO-R) on CD4+ T cells directly inhibits TH17 generation and promotes trans-differentiation of TH17 into IL-17-FOXP3+CD4+ T cells. Mechanistically, EPO/EPO-R ligation abrogates upregulation of SGK1 gene expression and blocks p38 activity to prevent SGK1 phosphorylation, thereby inhibiting RORC-mediated transcription of IL-17 and IL-23 receptor genes. In a murine model of TH17-dependent aristolochic acid (ArA)-induced, interstitial kidney disease associated with reduced renal EPO production, we demonstrate that transgenic EPO overexpression or recombinant EPO (rEPO) administration limits TH17 formation and clinical/histological disease expression. EPO/EPO-R ligations on CD4+ T cells abrogate, while absence of T cell-expressed EPO-R augments, TH17 induction and clinical/histological expression of pristane-induced glomerulonephritis (associated with decreased intrarenal EPO). rEPO prevents spontaneous glomerulonephritis and TH17 generation in MRL-lpr mice. Together, our findings indicate that EPO physiologically and therapeutically modulate TH17 cells to limit expression of TH17-associated autoimmune kidney disease.
IL-17 产生的 CD4+ 细胞(TH17)与自身免疫有关,包括自身免疫性肾病。促红细胞生成素(EPO)新发现的免疫调节功能及其主要的肾内来源表明,EPO 生理性地调节 TH17 分化,从而成为自身免疫性肾病发展的障碍。通过对人和鼠细胞的体外研究和体内模型的研究,我们表明,EPO 与其受体(EPO-R)在 CD4+T 细胞上的结合直接抑制 TH17 的产生,并促进 TH17 向 IL-17-FOXP3+CD4+T 细胞的转分化。从机制上讲,EPO/EPO-R 的结合可阻断 SGK1 基因表达的上调,并阻止 p38 活性,从而防止 SGK1 磷酸化,从而抑制 RORC 介导的 IL-17 和 IL-23 受体基因的转录。在一种依赖于 TH17 的马兜铃酸(ArA)诱导的间质性肾病的小鼠模型中,与肾内 EPO 产生减少有关,我们证明,转基因 EPO 过表达或重组 EPO(rEPO)给药可限制 TH17 的形成和临床/组织学疾病表现。EPO/EPO-R 在 CD4+T 细胞上的结合可消除,而 T 细胞表达的 EPO-R 的缺失则增强了 pristane 诱导的肾小球肾炎(与肾内 EPO 减少有关)的 TH17 诱导和临床/组织学表达。rEPO 可预防自发性肾小球肾炎和 MRL-lpr 小鼠中 TH17 的产生。总之,我们的研究结果表明,EPO 可生理性和治疗性地调节 TH17 细胞,以限制与 TH17 相关的自身免疫性肾病的表达。