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

J Exp Med. 2005 Aug 15;202(4):473-7. doi: 10.1084/jem.20050304. Epub 2005 Aug 8.

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

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Aquaporin 4
  • Aquaporins / genetics
  • Aquaporins / immunology*
  • Astrocytes / immunology
  • Astrocytes / pathology
  • Autoantibodies / blood
  • Autoantibodies / immunology*
  • Biomarkers / blood
  • Blood-Brain Barrier / immunology
  • Blood-Brain Barrier / metabolism
  • Dystroglycans / immunology
  • Humans
  • Immunoglobulin G / blood
  • Immunoglobulin G / immunology*
  • Mice
  • Mice, Mutant Strains
  • Multiprotein Complexes / genetics
  • Multiprotein Complexes / immunology*
  • Neuromyelitis Optica / blood
  • Neuromyelitis Optica / immunology*
  • Neuromyelitis Optica / pathology
  • Optic Nerve / immunology
  • Optic Nerve / pathology
  • Protein Binding / immunology
  • Rats
  • Spinal Cord / immunology
  • Spinal Cord / pathology

Substances

  • AQP4 protein, human
  • Aqp4 protein, mouse
  • Aqp4 protein, rat
  • Aquaporin 4
  • Aquaporins
  • Autoantibodies
  • Biomarkers
  • Immunoglobulin G
  • Multiprotein Complexes
  • Dystroglycans