Abstract
Neuromyelitis optica (NMO) is an autoantibody-triggered neuro-inflammatory disease which preferentially attacks the spinal cord and optic nerve. Its defining autoantibody is specific for the water channel protein, aquaporin‐4 (AQP4), which primarily is localized at the end-feet of astrocytes. Histopathology studies of early NMO lesions demonstrated prominent activation of microglia, the resident immune sentinels of the central nervous system (CNS). Significant microglial reactivity is also observed in NMO animal models induced by introducing AQP4-IgG into the CNS. Here we review the potential roles for microglial activation in human NMO patients as well as different animal models of NMO. We will focus primarily on the molecular mechanisms underlying microglial function and microglia-astrocyte interaction in NMO pathogenesis. Understanding the role of microglia in NMO pathology may yield novel therapeutic approaches for this disease.
Highlights
Neuromyelitis optica (NMO) is an autoimmune inflammatory disorder of the central nervous system (CNS) which mainly affects optic nerves and the spinal cord [1]
NMO was largely considered a subtype of multiple sclerosis (MS), until 2004 when Lennon and colleagues identified that autoantibodies directed towards aquaporin‐4 (AQP4-IgG, called NMO-IgG) were a biomarker for NMO pathology [4]
To develop microglia specific treatments, future studies are needed to further our understanding of the molecular mechanisms underlying the role of microglia in NMO
Summary
Neuromyelitis optica (NMO) is an autoimmune inflammatory disorder of the central nervous system (CNS) which mainly affects optic nerves and the spinal cord [1]. In addition to loss of AQP4 and astrocytes, neuronal injury, demyelination, microglial activation, and macrophage infiltration are prominent in AQP4-IgG seropositive NMO pathology [14]. Consistent with the role of complement in microglial function, microglia are able to secret complement components to activate astrocytes and mediate neuronal injury in a variety of neurological disorders [33,34,35]. Some recent rodent models of NMO have utilized passive transfer of AQP4-IgG into animals to induce clinically relevant pathology and provide early evidence of microglial activation in NMO pathology [58, 61, 71].
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