Abstract
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.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Animals
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Aquaporin 4
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Aquaporins / genetics
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Aquaporins / immunology*
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Astrocytes / immunology
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Astrocytes / pathology
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Autoantibodies / blood
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Autoantibodies / immunology*
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Biomarkers / blood
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Blood-Brain Barrier / immunology
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Blood-Brain Barrier / metabolism
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Dystroglycans / immunology
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Humans
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Immunoglobulin G / blood
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Immunoglobulin G / immunology*
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Mice
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Mice, Mutant Strains
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Multiprotein Complexes / genetics
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Multiprotein Complexes / immunology*
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Neuromyelitis Optica / blood
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Neuromyelitis Optica / immunology*
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Neuromyelitis Optica / pathology
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Optic Nerve / immunology
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Optic Nerve / pathology
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Protein Binding / immunology
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Rats
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Spinal Cord / immunology
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Spinal Cord / pathology
Substances
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AQP4 protein, human
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Aqp4 protein, mouse
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Aqp4 protein, rat
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Aquaporin 4
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Aquaporins
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Autoantibodies
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Biomarkers
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Immunoglobulin G
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Multiprotein Complexes
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Dystroglycans