Mechanism of disease


Within the central nervous system (CNS), AQP4 is expressed in the foot processes of the astrocytes lining the blood–brain barrier (BBB).2 The pathogenic AQP4‑IgG antibodies cross the BBB and bind to AQP4 antigens on the astrocytes, causing astrocytic destruction and eventually demyelination and nerve damage via complement-mediated cytotoxicity and inflammation.1–3
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AQP4, aquaporin-4; BBB, blood–brain barrier; C5, complement component 5; CNS, central nervous system; IgG, immunoglobulin G; NMOSD, neuromyelitis optica spectrum disorder.
Jarius S, et al. Neuromyelitis optica. Nat Rev Dis Primers. 2020;6(1):85. Dalakas MC, Alexopoulos H, Spaeth PJ. Complement in neurological disorders and emerging complement-targeted therapeutics. Nat Rev Neurol. 2020;16(11):601–617. Dutra BG, et al. Neuromyelitis optica spectrum disorders: Spectrum of MR imaging findings and their differential diagnosis. Radiographics. 2018;38(1):169–193. advance I am a healthcare professional registered in the EU I am not a
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