St George's, University of London, London, UK.
Department of Obstetrics and Gynaecology, University of British Columbia and BC Children's Hospital Research Institute, Vancouver, BC, Canada.
Neuropathol Appl Neurobiol. 2020 Apr;46(3):199-218. doi: 10.1111/nan.12574. Epub 2019 Aug 14.
Neuromyelitis optica is an autoimmune inflammatory disorder of the central nervous system that preferentially targets the spinal cord and optic nerve. Following the discovery of circulating antibodies against the astrocytic aquaporin 4 (AQP4) water channel protein, recent studies have expanded our knowledge of the unique complexities of the pathogenesis of neuromyelitis optica and its relationship with the immune response. This review describes and summarizes the recent advances in our understanding of the molecular mechanisms underlying neuromyelitis optica disease pathology and examines their potential as therapeutic targets. Additionally, we update the most recent research by proposing major unanswered questions regarding how peripheral AQP4 antibodies are produced and their entry into the central nervous system, the causes of AQP4-IgG-seronegative disease, why peripheral AQP4-expressing organs are spared from damage, and the impact of this disease on pregnancy.
视神经脊髓炎是一种中枢神经系统自身免疫性炎症性疾病，主要累及脊髓和视神经。随着针对星形胶质细胞水通道蛋白 4（AQP4）水通道蛋白的循环抗体的发现，最近的研究扩展了我们对视神经脊髓炎发病机制的独特复杂性及其与免疫反应关系的认识。本综述描述并总结了我们对视神经脊髓炎疾病病理学潜在分子机制的理解的最新进展，并研究了它们作为治疗靶点的潜力。此外，我们通过提出有关外周 AQP4 抗体产生及其进入中枢神经系统的主要未解决问题、AQP4-IgG 血清阴性疾病的原因、为什么外周表达 AQP4 的器官免受损伤以及该疾病对妊娠的影响等问题，更新了最近的研究进展。
