title: Review of Animal Models of Neuromyelitis Optica.
abstract: Neuromyelitis optica (NMO) is a recurrent neuroinflammatory disease of the optic nerves and spinal cord associated with the anti-aquaporin-4 (AQP4) antibody biomarker, NMO-IgG. As clinical and scientific research interest in NMO grows, the need for an animal model becomes more urgent. Over the past few years, several groups have developed rodent models that partially represent human NMO disease. Passive transfer of the NMO-IgG is not pathogenic alone, but in certain contexts can recruit granulocytes and lead to increased inflammation. Studies of the cellular immune response against AQP4 have also shed light on the roles of B and T cells in NMO, especially focusing on the role of Th17 T helper cells. This review discusses the contribution of the available NMO animal models to the understanding of NMO disease pathogenesis.
authors.affiliation: Department of Neurology, Johns Hopkins University, Baltimore, MD, USA.
authors.forename: Melina V
authors.initials: MV
authors.lastname: Jones
authors.affiliation: Department of Neurology, University Hospitals of Strasbourg, Strasbourg, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Collongues
authors.affiliation: Department of Neurology, University Hospitals of Strasbourg, Strasbourg, France.
authors.forename: Jerome
authors.initials: J
authors.lastname: de Seze
authors.affiliation: Department of Neurology, Department of Microbiology and Immunology, Osaka University, Osaka, Japan.
authors.forename: Makoto
authors.initials: M
authors.lastname: Kinoshita
authors.affiliation: Department of Neurology, Osaka University, Osaka, Japan.
authors.forename: Yuji
authors.initials: Y
authors.lastname: Nakatsuji
authors.affiliation: Department of Neurology, Johns Hopkins University, Baltimore, MD, USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Levy
keywords: Aquaporin-4; Experimental Autoimmune Encephalomyelitis; NMO-IgG; Neuromyelitis optica