The Journal of Experimental Medicine · 2005 · 2.3K citations · 26 references
Life SciencesNeurologyNeuroscienceAutoimmunityNeuroimmunologyNeuropathologyAutoimmune Neurological DisordersChannelopathiesDemyelinating DiseasesDemyelinating DisordersMultiple SclerosisNeuroinflammationAquaporin DisordersAquaporin TransportAquaporin-4 Antibody DiseaseAquaporin-mediated Water TransportDemyelination PathophysiologyDystroglycan ComplexImmune-mediated DemyelinationSerum Autoantibody Testing
Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that selectively affects optic nerves and spinal cord. It is considered a severe variant of multiple sclerosis (MS), and frequently is misdiagnosed as MS, but prognosis and optimal treatments differ. A serum immunoglobulin G autoantibody (NMO-IgG) serves as a specific marker for NMO. Here we show that NMO-IgG binds selectively to the aquaporin-4 water channel, a component of the dystroglycan protein complex located in astrocytic foot processes at the blood-brain barrier. NMO may represent the first example of a novel class of autoimmune channelopathy.
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A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis
Vanda A. Lennon, Dean M. Wingerchuk, Thomas J. Kryzer et al. · The Lancet · 2004 · 3.2K citations
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The clinical course of neuromyelitis optica (Devic’s syndrome)
Dean M. Wingerchuk, William F. Hogancamp, Peter C. O’Brien et al. · Neurology · 1999 · 1.8K citations
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Annabel M. Ruiter, Rosemarijn R. J. Somanje-Bolweg, Erik van Gorsel et al. · PubMed · 2017 · 1.8K citations · Full text
Aquaporin-4 deletion in mice reduces brain edema after acute water intoxication and ischemic stroke
Geoffrey T. Manley, Miki Fujimura, Tonghui Ma et al. · Nature Medicine · 2000 · 1.5K citations
Medicine, Neurology, Neuroscience +17