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Phenotype analysis of <jats:styled-content style=\"fixed-case\">AQP<\/jats:styled-content>4 knockout mice indicates the involvement of <jats:styled-content style=\"fixed-case\">AQP<\/jats:styled-content>4 in water movement into and out of the brain, astrocyte migration, glial scar formation and neuroexcitatory phenomena. <jats:styled-content style=\"fixed-case\">AQP<\/jats:styled-content>4 monomers form tetramers in membranes, which further aggregate to form supramolecular assemblies called orthogonal arrays of particles. <jats:styled-content style=\"fixed-case\">AQP<\/jats:styled-content>4\u2010<jats:styled-content style=\"fixed-case\">IgG<\/jats:styled-content> is pathogenic in <jats:styled-content style=\"fixed-case\">NMO<\/jats:styled-content> by a mechanism involving complement\u2010 and cell\u2010mediated astrocyte cytotoxicity, which produces an inflammatory response with oligodendrocyte injury and demyelination. <jats:styled-content style=\"fixed-case\">AQP<\/jats:styled-content>4 orthogonal arrays are crucial in <jats:styled-content style=\"fixed-case\">NMO<\/jats:styled-content> pathogenesis, as they increase <jats:styled-content style=\"fixed-case\">AQP<\/jats:styled-content>4\u2010<jats:styled-content style=\"fixed-case\">IgG<\/jats:styled-content> binding to <jats:styled-content style=\"fixed-case\">AQP<\/jats:styled-content>4 and greatly enhance complement\u2010dependent cytotoxicity. Novel <jats:styled-content style=\"fixed-case\">NMO<\/jats:styled-content> therapeutics are under development that target <jats:styled-content style=\"fixed-case\">AQP<\/jats:styled-content>4\u2010<jats:styled-content style=\"fixed-case\">IgG<\/jats:styled-content> or <jats:styled-content style=\"fixed-case\">AQP<\/jats:styled-content>4, including aquaporumab monoclonal antibodies and small molecules that block <jats:styled-content style=\"fixed-case\">AQP<\/jats:styled-content>4\u2010<jats:styled-content style=\"fixed-case\">IgG<\/jats:styled-content> binding to <jats:styled-content style=\"fixed-case\">AQP<\/jats:styled-content>4, and enzymatic inactivation strategies to neutralize <jats:styled-content style=\"fixed-case\">AQP<\/jats:styled-content>4\u2010<jats:styled-content style=\"fixed-case\">IgG<\/jats:styled-content> pathogenicity.<\/jats:p>","DOI":"10.1111\/bpa.12085","type":"journal-article","created":{"date-parts":[[2013,10,14]],"date-time":"2013-10-14T06:19:27Z","timestamp":1381731567000},"page":"684-695","source":"Crossref","is-referenced-by-count":110,"title":["Biology of <scp>AQP4<\/scp> and Anti\u2010<scp>AQP4<\/scp> Antibody: Therapeutic Implications for <scp>NMO<\/scp>"],"prefix":"10.1111","volume":"23","author":[{"given":"A. 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