The role of AQP4 in neuromyelitis optica: More answers, more questions.
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.
Department of Neurology & Institute of Neurology, Ruijin Hospital Affiliated to Shanghai Jiaotong University, School of Medicine, Shanghai 200025, China.
Xin
X
Yang
Neurology Department, University of Washington School of Medicine, Seattle, Washington, 98039, USA.
Bruce R
BR
Ransom
Department of Neurology & Institute of Neurology, Ruijin Hospital Affiliated to Shanghai Jiaotong University, School of Medicine, Shanghai 200025, China. Electronic address: majifa@hotmail.com.
Jian-Fang
JF
Ma
D000818:Animals; D051401:Aquaporin 4 / Q000378:metabolism*; D002490:Central Nervous System / Q000378:metabolism*; D018698:Glutamic Acid / Q000378:metabolism; D006801:Humans; D009471:Neuromyelitis Optica / Q000378:metabolism* / Q000473:pathology*; D011188:Potassium / Q000378:metabolism
D051401:Aquaporin 4; D018698:Glutamic Acid; D011188:Potassium
Complement; Demyelination; Glutamate; Neuromyelitis optica; Potassium channel; Water channel protein aquaporin-4