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

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)
The Journal of Experimental Medicine
August 8, 2005
Cited by 2,265Open Access
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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.


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