Clarification of aquaporin-4 related molecular and cellular pathomechanism in neuromyelitis optica (Devic's disease)

视神经脊髓炎(NMO)是一种以反复发作的严重视神经炎和横贯性脊髓炎为特征的难治性神经系统疾病。本研究表明，水通道蛋白4(AQP4)抗体是NMO特有的自身抗体，它的滴度反映了疾病的活动性。我们还证明了AQP4抗体在体外和体内都具有致病潜力，NMO是一种新的临床实体(免疫介导的星形细胞病变)。关于NMO的治疗，我们发现长期逐步减少类固醇在预防该病早期复发方面的疗效。

Neuromyelitis optica (NMO)is an intractable neurologic disease characterized by recurrent severe optic neuritis and transverse myelitis. The present study showed that the titers of aquaporin 4 (AQP4)antibody, an autoantibody unique to NMO, reflect disease activity. We also demonstrated that AQP4 antibody has a pathogenic potential in vitro and in vivo, and that NMO is a new clinical entity (immune-mediated astrocytopathy). Concerning therapy of NMO, we found therapeutic efficacy of long-term gradual steroid tapering in preventing early relapse of this disease.