The Journal of Experimental Medicine
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Published online 8 August 2005 doi:10.1084/jem.20050304
Rockefeller University Press, 0022-1007 $8.00
JEM, Volume 202, Number 4, 473-477
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BRIEF DEFINITIVE REPORT

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

Vanda A. Lennon1,2,3, Thomas J. Kryzer3, Sean J. Pittock2,3, A.S. Verkman4, and Shannon R. Hinson3

1 Department of Immunology, Mayo Clinic College of Medicine, Rochester, MN 55905
2 Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN 55905
3 Department of Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN 55905
4 Cardiovascular Research Institute, University of California, San Francisco, CA 94143

CORRESPONDENCE Vanda A. Lennon: lennon.vanda{at}mayo.edu

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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