Misu Tatsuro
Nihon Rinsho. 2014 Nov;72(11):1941-8.
Neuromyelitis optica (NMO) is autoimmune disease targeting against water channel aquaporin 4 (AQP4), mainly located at astrocyte foot processes in the central nervous system (CNS). The basis of NMO pathology is the astrocytopathy, but it is recently revealed that there is the diversity of NMO lesion possibly links to the mechanism of astrocyte death with and without complement. In vitro and in vivo models of NMO suggested that this autoantibody is pathogenic. There are mainly two types of in vivo experimental rodent models of NMO, one is a model of direct intracranial injection of NMO-IgG and the other is a passive transfer of NMO-IgG in experimental autoimmune encephalomyelitis (EAE). Both is useful to study the pathogenicity of NMO-IgG, and is promising to create the new generation therapy for autoimmune CNS diseases.
视神经脊髓炎（NMO）是一种针对水通道蛋白4（AQP4）的自身免疫性疾病，AQP4主要位于中枢神经系统（CNS）的星形胶质细胞足突。NMO病理学的基础是星形胶质细胞病变，但最近发现NMO病变的多样性可能与有或无补体情况下星形胶质细胞死亡的机制有关。NMO的体外和体内模型表明这种自身抗体具有致病性。NMO的体内实验啮齿动物模型主要有两种类型，一种是直接颅内注射NMO-IgG的模型，另一种是在实验性自身免疫性脑脊髓炎（EAE）中被动转移NMO-IgG的模型。这两种模型都有助于研究NMO-IgG的致病性，并且有望为自身免疫性中枢神经系统疾病创造新一代的治疗方法。
