2022 年 39 巻 3 号 p. 251-256
Neuromyelitis optica (NMO) is a disease characterized by severe optic neuritis and transverse myelitis with autoantibody against aquaporin 4 (AQP4), mainly localized at astrocyte foot processes. Loss of AQP4 and glial fibrillary acidic protein with relatively preserved myelin is the pathological hallmark of active NMO lesions. Several experimental studies suggested the crucial role of AQP4 antibody with diverse mechanisms of tissue injury in NMOSD including antibody– and complement–induced cytotoxicity against astrocytes. This diversity includes several conditions including astrocyte lysis, sub–lysis, clasmatodendrosis, reactive gliosis, and altered expression of other gap junction proteins such as connexin. The damage of glia–neuron networks related to astrocytes could induce the secondary tissue injury such as demyelination and axonal loss.
