The role of AQP4 in neuromyelitis optica: More answers, more questions

J Neuroimmunol. 2016 Sep 15:298:63-70. doi: 10.1016/j.jneuroim.2016.06.002. Epub 2016 Jun 7.

Abstract

Neuromyelitis optica (NMO) is a recurrent inflammatory disease that preferentially targets the optic nerves and spinal cord. The presence of antibodies to the water channel protein aquaporin-4 (AQP4), expressed almost exclusively in astrocytes in the central nervous system (CNS), is a reliable biomarker for NMO. These antibodies, NMO-IgG, may be responsible for the sequential cascade of immune events, including IgG/IgM deposition, infiltration of granulocytes and complement-mediated cytotoxicity (i.e. astrocyte loss) and demyelination. This review summarizes current thinking about the role of NMO-IgG in the pathogenesis of this condition. New insights were also generated along with important additional questions.

Keywords: Complement; Demyelination; Glutamate; Neuromyelitis optica; Potassium channel; Water channel protein aquaporin-4.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Aquaporin 4 / metabolism*
  • Central Nervous System / metabolism*
  • Glutamic Acid / metabolism
  • Humans
  • Neuromyelitis Optica / metabolism*
  • Neuromyelitis Optica / pathology*
  • Potassium / metabolism

Substances

  • Aquaporin 4
  • Glutamic Acid
  • Potassium