IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel.
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.
Lennon
Vanda A
VA
Department of Immunology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA. lennon.vanda@mayo.edu
Kryzer
Thomas J
TJ
Pittock
Sean J
SJ
Verkman
A S
AS
Hinson
Shannon R
SR
D000818:Animals; D051401:Aquaporin 4; D020346:Aquaporins / Q000235:genetics / Q000276:immunology*; D001253:Astrocytes / Q000276:immunology / Q000473:pathology; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D015415:Biomarkers / Q000097:blood; D001812:Blood-Brain Barrier / Q000276:immunology / Q000378:metabolism; D049030:Dystroglycans / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D051379:Mice; D008817:Mice, Mutant Strains; D046912:Multiprotein Complexes / Q000235:genetics / Q000276:immunology*; D009471:Neuromyelitis Optica / Q000097:blood / Q000276:immunology* / Q000473:pathology; D009900:Optic Nerve / Q000276:immunology / Q000473:pathology; D011485:Protein Binding / Q000276:immunology; D051381:Rats; D013116:Spinal Cord / Q000276:immunology / Q000473:pathology
C496030:AQP4 protein, human; C496031:Aqp4 protein, mouse; C496032:Aqp4 protein, rat; D051401:Aquaporin 4; D020346:Aquaporins; D001323:Autoantibodies; D015415:Biomarkers; D007074:Immunoglobulin G; D046912:Multiprotein Complexes; D049030:Dystroglycans