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. 2021 Jun 16;18(14):3059–3065. doi: 10.7150/ijms.61153

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Factors secreted by activated microglia are involved in the pathogenesis of NMOSD. NMO-IgG binds to AQP4 on astrocytes, resulting in astrocyte activation. Activated astrocytes secrete complement C3a, which binds with C3aR on resting microglia, leading to microglial activation. The secretion of complement C1q, inflammatory cytokines and PGRN by activated microglia is increased. These secretions further promote microglial activation in an autocrine manner and facilitate localized injury to neurons, astrocytes and oligodendrocytes in a paracrine manner. AQP4, aquaporin 4; NMO-IgG, neuromyelitis optica immunoglobulin; PGRN, progranulin.