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. Author manuscript; available in PMC: 2017 Jan 22.
Published in final edited form as: Nat Rev Neurosci. 2015 May;16(5):249–263. doi: 10.1038/nrn3898

Figure 4. Model of NMO as an astrocytopathy with causal contributions of astrocyte gain- and loss- of functions to different stages of disease progression.

Figure 4

a| Stage 1. Sporadic or disease specific breaches of the blood-brain barrier lead to binding of AQP4/NMO-IgG to astrocyte membranes, which results in astrocyte functional changes. b| Stage 2. AQP4/NMO-IgG binding leads to astrocyte production of pro-inflammatory molecules and recruitment of leukocytes, initially into perivascular spaces (PVS). This process may be exacerbated by concomitant exposure to inflammatory mediators deriving from local or peripheral infections, such as DAMPs, PAMPs or INFγ. This process may be attenuated by estrogen related signaling mechanisms. c| Stage 3. Compliment-mediated astrocyte destruction leads to loss of astrocyte barrier functions and subsequent spread of neurotoxic inflammation into adjacent neural parenchyma.