1Department of Neurology, Tianjin Neurological Institute, Tianjin Medical University General Hospital, Tianjin 300052, China
2Department of Neurology, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, P hoenix, AZ 85013, USA
Corresponding author: Yaping Yan. E-mail: yaping.yan@tijmu.edu.cn

Abstract 

Neuromyelitis optica (NMO) is a recurrent inflammatory disease that predominantly attacks the opticnerves and spinal cord. NMO-IgG, the specific autoantibody present in the vast majority of NMO patients, targets the astrocytic water channel protein aquaporin 4 (AQP4), and differentiates NMO from multiple sclerosis. The growing clinical and research interest in NMO makes it urgent to produce an animal model of NMO. The pathogenic effect of anti-AQP4 antibodies derived from the serum of patients paves the way to generating an experimental model based on the anti-AQP4-mediated astrocyte damage. In this review, we discuss the contribution of experimental models to the understanding of the pathogenesis of the disease and drug development. Key questions raised by the existing models are also discussed.

Keywords

n euromyelitis optica; animal model; NMO-IgG; astrocyte

[SpringerLink]