title: Mechanisms of disease: aquaporin-4 antibodies in neuromyelitis optica.
abstract: Neuromyelitis optica (NMO) is a rare CNS inflammatory disorder that predominantly affects the optic nerves and spinal cord. Recent serological findings strongly suggest that NMO is a distinct disease rather than a subtype of multiple sclerosis. In NMO, serum antibodies, collectively known as NMO-IgG, characteristically bind to cerebral microvessels, pia mater and Virchow-Robin spaces. The main target antigen for this immunoreactivity has been identified as aquaporin-4 (AQP4). The antibodies are highly specific for NMO, and they are also found in patients with longitudinally extensive transverse myelitis without optic neuritis, which is thought to be a precursor to NMO in some cases. An antibody-mediated pathogenesis for NMO is supported by several observations, including the characteristics of the AQP4 antibodies, the distinct NMO pathology--which includes IgG and complement deposition and loss of AQP4 from spinal cord lesions--and emerging evidence of the beneficial effects of B-cell depletion and plasma exchange. Many aspects of the pathogenesis, however, remain unclear.
authors.affiliation: Weatherall Institute of Molecular Medicine, University of Oxford, UK.
authors.forename: Sven
authors.initials: S
authors.lastname: Jarius
authors.forename: Friedemann
authors.initials: F
authors.lastname: Paul
authors.forename: Diego
authors.initials: D
authors.lastname: Franciotta
authors.forename: Patrick
authors.initials: P
authors.lastname: Waters
authors.forename: Frauke
authors.initials: F
authors.lastname: Zipp
authors.forename: Reinhard
authors.initials: R
authors.lastname: Hohlfeld
authors.forename: Angela
authors.initials: A
authors.lastname: Vincent
authors.forename: Brigitte
authors.initials: B
authors.lastname: Wildemann
mesh_terms: D000906:Antibodies / Q000097:blood*; D051401:Aquaporin 4 / Q000276:immunology* / Q000378:metabolism; D001666:Binding Sites, Antibody; D006801:Humans; D009471:Neuromyelitis Optica / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology
chemical_list: D000906:Antibodies; D051401:Aquaporin 4