{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T07:29:33Z","timestamp":1793172573621,"version":"4.5.0"},"reference-count":45,"publisher":"National Academy of Sciences","issue":"6","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2007,2,6]]},"abstract":"<jats:p>\n                    In humans, mutations in the X-linked\n                    <jats:italic>MECP2<\/jats:italic>\n                    gene, are the cause of Rett syndrome (RTT), a neurodevelopmental disorder that affects mainly girls. MeCP2 binds to methylated CpGs and is thought to act as a transcriptional repressor. In male mice, deletion or targeted mutation of\n                    <jats:italic>Mecp2<\/jats:italic>\n                    leads to lethality and causes a neuronal phenotype. Selective mutation of\n                    <jats:italic>Mecp2<\/jats:italic>\n                    in postnatal neurons results in a similar, although delayed, phenotype, suggesting that the symptoms are caused by MeCP2 deficiency in postmitotic neurons. In agreement with this idea, expression of a Mecp2 transgene in postmitotic neurons of\n                    <jats:italic>Mecp2<\/jats:italic>\n                    -null mutant mice resulted in the phenotypical rescue of the symptoms. To assess whether postnatal activation of MeCP2 in mutant animals could also affect the progression of the disorder, we constructed a conditionally active Mecp2 \u201crescue transgene\u201d that was activated between P0 and P30. The Mecp2 transgene was under the control of the CAGGS promoter and was activated by using brain specific Cre-mediated recombination. Our results indicate that postnatal, neuron-specific activation of MeCP2 as late as 2\u20134 weeks of age significantly prolonged the lifespan of mutant animals and delayed the onset of neurologic symptoms.\n                  <\/jats:p>","DOI":"10.1073\/pnas.0610593104","type":"journal-article","created":{"date-parts":[[2007,2,2]],"date-time":"2007-02-02T22:57:49Z","timestamp":1170457069000},"page":"1931-1936","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":237,"title":["Partial rescue of MeCP2 deficiency by postnatal activation of MeCP2"],"prefix":"10.1073","volume":"104","author":[{"given":"Emanuela","family":"Giacometti","sequence":"first","affiliation":[{"name":"Whitehead Institute for Biomedical Research, 9 Cambridge Center, Cambridge, MA 02142; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Luikenhuis","sequence":"additional","affiliation":[{"name":"Whitehead Institute for Biomedical 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