title: Experimental models of neuromyelitis optica: current status, challenges and future directions.
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.
authors.affiliation: Department of Neurology, Tianjin Neurological Institute, Tianjin Medical University General Hospital, Tianjin, 300052, China.|Department of Neurology, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ, 85013, USA.
authors.forename: Minshu
authors.initials: M
authors.lastname: Li
authors.affiliation: Department of Neurology, Tianjin Neurological Institute, Tianjin Medical University General Hospital, Tianjin, 300052, China. yaping.yan@tijmu.edu.cn.
authors.forename: Yaping
authors.initials: Y
authors.lastname: Yan
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D006801:Humans; D009471:Neuromyelitis Optica
keywords: NMO-IgG; animal model; astrocyte; neuromyelitis optica