Matta Bharati, Song Su, Li Dan, Barnes Betsy J
Center for Autoimmune and Musculoskeletal Diseases, The Feinstein Institute for Medical Research, Manhasset, NY 11030, United States.
Center for Autoimmune and Musculoskeletal Diseases, The Feinstein Institute for Medical Research, Manhasset, NY 11030, United States.
Cytokine. 2017 Oct;98:15-26. doi: 10.1016/j.cyto.2017.02.006. Epub 2017 Mar 7.
Interferon regulatory factors (IRFs) play critical roles in pathogen-induced innate immune responses and the subsequent induction of adaptive immune response. Dysregulation of IRF signaling is therefore thought to contribute to autoimmune disease pathogenesis. Indeed, numerous murine in vivo studies have documented protection from or enhanced susceptibility to particular autoimmune diseases in Irf-deficient mice. What has been lacking, however, is replication of these in vivo observations in primary immune cells from patients with autoimmune disease. These types of studies are essential as the majority of in vivo data support a protective role for IRFs in Irf-deficient mice, yet IRFs are often found to be overexpressed in patient immune cells. A significant body of work is beginning to emerge from both of these areas of study - mouse and human.
干扰素调节因子（IRFs）在病原体诱导的先天性免疫反应以及随后适应性免疫反应的诱导中发挥关键作用。因此，IRF信号失调被认为与自身免疫性疾病的发病机制有关。事实上，大量的小鼠体内研究已经证明，Irf缺陷小鼠对特定自身免疫性疾病具有保护作用或易感性增强。然而，缺乏的是在自身免疫性疾病患者的原代免疫细胞中重复这些体内观察结果。这类研究至关重要，因为大多数体内数据支持IRFs在Irf缺陷小鼠中具有保护作用，但在患者免疫细胞中常常发现IRFs过度表达。来自小鼠和人类这两个研究领域的大量工作开始涌现。
