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Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2018,8,7]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>\n                    Rett syndrome (RTT) is an X-linked neurodevelopmental disorder caused by mutations in\n                    <jats:italic>MECP2<\/jats:italic>\n                    . To treat RTT, reactivating the dormant copy of\n                    <jats:italic>MECP2<\/jats:italic>\n                    on the inactive X has been of considerable interest. Although potential therapeutics have been identified, their development has been hampered by the lack of a suitable female mouse model and uncertainty regarding how much MECP2 needs to be restored. Here, we create a female model with a more severe phenotype, including a short lifespan, neuromotor impairment, and repetitive behaviors often seen in human RTT. Significantly, 5\u201310% MECP2 restoration in brain extends lifespan by eightfold and improves RTT phenotypes. Our study thus provides a much-needed female model and implies potential therapeutic benefit with low-level MECP2 expression.\n                  <\/jats:p>","DOI":"10.1073\/pnas.1800931115","type":"journal-article","created":{"date-parts":[[2018,7,23]],"date-time":"2018-07-23T15:16:36Z","timestamp":1532358996000},"page":"8185-8190","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":43,"title":["Tsix\u2013Mecp2 female mouse model for Rett syndrome reveals that low-level MECP2 expression extends life and improves neuromotor function"],"prefix":"10.1073","volume":"115","author":[{"given":"Lieselot L. 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