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IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel

The Journal of Experimental Medicine · 2005 · Vol. 202(4) · pp. 473–477

Vanda A. Lennon✉(Mayo Clinic)Thomas J. Kryzer(Mayo Clinic)Sean J. Pittock(Mayo Clinic)A. S. Verkman(University of California, San Francisco)Shannon R. Hinson(Mayo Clinic)

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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.

Multiple Sclerosis Research Studies Peripheral Neuropathies and Disorders Sphingolipid Metabolism and SignalingNeuromyelitis opticaMultiple sclerosisAquaporin 4ChannelopathyAutoantibodyOptic neuritisOptic nerveMedicineWater channelSpinal cord

MeSH terms

AnimalsAstrocytesAutoantibodiesBlood-Brain BarrierHumansImmunoglobulin GMice, Mutant StrainsNeuromyelitis OpticaOptic NerveProtein BindingSpinal CordBiomarkersAquaporinsMultiprotein ComplexesDystroglycans

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