Department Neuroimmunology, Center for Brain Research, Medical University Vienna, Vienna, Austria.
Brain Pathol. 2014 Jan;24(1):74-82. doi: 10.1111/bpa.12098.
For a long time, the most important inflammatory demyelinating diseases of the central nervous system (CNS), for example, multiple sclerosis (MS) and neuromyelitis optica (NMO), were extremely hard to differentiate, often with severe consequences for affected patients. This changed with the discovery of NMO-immunoglobulin G (IgG), a specific autoantibody which was detected in the vast majority of NMO patients, and with the demonstration that this autoantibody targets aquaporin 4 (AQP4), a water channel found on astrocytes in the CNS. These findings paved the way for the generation of experimental models of NMO. This chapter will discuss the contribution of experimental models to NMO research and what key questions remain to be addressed.
长期以来，中枢神经系统（CNS）中最重要的炎症性脱髓鞘疾病，例如多发性硬化症（MS）和视神经脊髓炎（NMO），极难区分，这对受影响的患者往往会产生严重后果。随着 NMO-免疫球蛋白 G（IgG）的发现，这种情况发生了变化，这种特异性自身抗体在绝大多数 NMO 患者中被检测到，并且表明该自身抗体靶向水通道蛋白 4（AQP4），AQP4 是中枢神经系统星形胶质细胞上的一种水通道。这些发现为 NMO 的实验模型的产生铺平了道路。本章将讨论实验模型对 NMO 研究的贡献，以及仍有待解决的关键问题。
