Blank Thomas, Goldmann Tobias, Koch Mirja, Amann Lukas, Schön Christian, Bonin Michael, Pang Shengru, Prinz Marco, Burnet Michael, Wagner Johanna E, Biel Martin, Michalakis Stylianos
Institute of Neuropathology, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
In Vivo Pharmacology, Synovo GmbH, Tübingen, Germany.
Front Immunol. 2018 Jan 5;8:1930. doi: 10.3389/fimmu.2017.01930. eCollection 2017.
Retinitis pigmentosa (RP) denotes a family of inherited blinding eye diseases characterized by progressive degeneration of rod and cone photoreceptors in the retina. In most cases, a rod-specific genetic defect results in early functional loss and degeneration of rods, which is followed by degeneration of cones and loss of daylight vision at later stages. Microglial cells, the immune cells of the central nervous system, are activated in retinas of RP patients and in several RP mouse models. However, it is still a matter of debate whether activated microglial cells may be responsible for the amplification of the typical degenerative processes. Here, we used mice, which represent a slow degenerative mouse model of RP, to investigate the extent of microglia activation in retinal degeneration. With a combination of FACS analysis, immunohistochemistry and gene expression analysis we established that microglia in the retina were already activated in an early, predegenerative stage of the disease. The evidence available so far suggests that early retinal microglia activation represents a first step in RP, which might initiate or accelerate photoreceptor degeneration.
视网膜色素变性（RP）是一类遗传性致盲眼病，其特征是视网膜中的视杆和视锥光感受器进行性退化。在大多数情况下，视杆特异性基因缺陷导致视杆早期功能丧失和退化，随后视锥退化，后期丧失明视觉。小胶质细胞是中枢神经系统的免疫细胞，在RP患者的视网膜和几种RP小鼠模型中被激活。然而，激活的小胶质细胞是否可能导致典型退化过程的放大仍存在争议。在这里，我们使用代表RP缓慢退化小鼠模型的小鼠，来研究视网膜退化中小胶质细胞激活的程度。通过流式细胞术分析、免疫组织化学和基因表达分析相结合，我们确定视网膜中的小胶质细胞在疾病的早期、预退化阶段就已经被激活。目前可得的证据表明，视网膜小胶质细胞的早期激活是RP的第一步，这可能引发或加速光感受器退化。
