Kavrochorianou Nadia, Evangelidou Maria, Markogiannaki Melina, Tovey Michael, Thyphronitis George, Haralambous Sylva
*Inflammation Research Group, Transgenic Technology Laboratory, Hellenic Pasteur Institute, Athens, Greece
†Laboratory of Molecular Genetics, Hellenic Pasteur Institute, Athens, Greece
J Leukoc Biol. 2016 Jan;99(1):175-88. doi: 10.1189/jlb.3A1214-598R. Epub 2015 Jul 31.
Although interferon-β is used as first-line therapy for multiple sclerosis, the cell type-specific activity of type I interferons in multiple sclerosis and its animal model, experimental autoimmune encephalomyelitis, remains obscure. In this study, we have elucidated the in vivo immunomodulatory role of type I interferon signaling in T cells during experimental autoimmune encephalomyelitis by use of a novel transgenic mouse, carrying a cd2-ifnar1 transgene on a interferon-α/β receptor 1 null genetic background, thus allowing expression of the interferon-α/β receptor 1 and hence, a functional type I interferon receptor exclusively on T cells. These transgenic mice exhibited milder experimental autoimmune encephalomyelitis with reduced T cell infiltration, demyelination, and axonal damage in the central nervous system. It is noteworthy that interferon-β administration in transgenic mice generated a more pronounced, protective effect against experimental autoimmune encephalomyelitis compared with untreated littermates. In vivo studies demonstrated that before experimental autoimmune encephalomyelitis onset, endogenous type I interferon receptor signaling in T cells led to impaired T-helper 17 responses, with a reduced fraction of CCR6(+) CD4(+) T cells in the periphery. At the acute phase, an increased proportion of interleukin-10- and interferon-γ-producing CD4(+) T cells was detected in the periphery of the transgenic mice, accompanied by up-regulation of the interferon-γ-induced gene Irgm1 in peripheral T cells. Together, these results reveal a hitherto unknown T cell-associated protective role of type I interferon in experimental autoimmune encephalomyelitis that may provide valuable clues for designing novel therapeutic strategies for multiple sclerosis.
尽管干扰素-β被用作多发性硬化症的一线治疗药物,但I型干扰素在多发性硬化症及其动物模型实验性自身免疫性脑脊髓炎中的细胞类型特异性活性仍不清楚。在本研究中,我们通过使用一种新型转基因小鼠阐明了I型干扰素信号在实验性自身免疫性脑脊髓炎期间T细胞中的体内免疫调节作用,该转基因小鼠在干扰素-α/β受体1基因缺失的遗传背景上携带cd2-ifnar1转基因,从而允许干扰素-α/β受体1的表达,进而仅在T细胞上表达功能性I型干扰素受体。这些转基因小鼠表现出较轻的实验性自身免疫性脑脊髓炎,中枢神经系统中的T细胞浸润、脱髓鞘和轴突损伤减少。值得注意的是,与未治疗的同窝小鼠相比,给转基因小鼠注射干扰素-β对实验性自身免疫性脑脊髓炎产生了更显著的保护作用。体内研究表明,在实验性自身免疫性脑脊髓炎发作之前,T细胞中的内源性I型干扰素受体信号导致辅助性T细胞17反应受损,外周CCR6(+) CD4(+) T细胞比例降低。在急性期,在转基因小鼠外周检测到产生白细胞介素-10和干扰素-γ的CD4(+) T细胞比例增加,同时外周T细胞中干扰素-γ诱导基因Irgm1上调。总之,这些结果揭示了I型干扰素在实验性自身免疫性脑脊髓炎中迄今未知的与T细胞相关的保护作用,这可能为设计多发性硬化症的新型治疗策略提供有价值的线索。