{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T12:16:14Z","timestamp":1792671374001,"version":"4.5.0"},"reference-count":32,"publisher":"National Academy of Sciences","issue":"16","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2004,4,20]]},"abstract":"<jats:p>\n                    Mutations in\n                    <jats:italic>MECP2<\/jats:italic>\n                    are the cause of Rett syndrome (RTT) in humans, a neurodevelopmental disorder that affects mainly girls. MeCP2 is a protein that binds CpG dinucleotides and is thought to act as a global transcriptional repressor. It is highly expressed in neurons, but not in glia, of the postnatal brain. The timing of MeCP2 activation correlates with the maturation of the central nervous system, and recent reports suggest that MeCP2 may be involved in the formation of synaptic contacts and may function in activity-dependent neuronal gene expression. Deletion or targeted mutation of\n                    <jats:italic>Mecp2<\/jats:italic>\n                    in mice leads to a Rett-like phenotype. Selective mutation of\n                    <jats:italic>Mecp2<\/jats:italic>\n                    in postnatal neurons leads to a similar, although delayed, phenotype, suggesting that MeCP2 plays a role in postmitotic neurons. Here we test the hypothesis that the symptoms of RTT are exclusively caused by a neuronal MeCP2 deficiency by placing\n                    <jats:italic>Mecp2<\/jats:italic>\n                    expression under the control of a neuron-specific promoter. Expression of the\n                    <jats:italic>Mecp2<\/jats:italic>\n                    transgene in postmitotic neurons resulted in symptoms of severe motor dysfunction. Transgene expression in\n                    <jats:italic>Mecp2<\/jats:italic>\n                    mutant mice, however, rescued the RTT phenotype.\n                  <\/jats:p>","DOI":"10.1073\/pnas.0401626101","type":"journal-article","created":{"date-parts":[[2004,4,6]],"date-time":"2004-04-06T22:12:53Z","timestamp":1081289573000},"page":"6033-6038","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":306,"title":["Expression of MeCP2 in postmitotic neurons rescues Rett syndrome in mice"],"prefix":"10.1073","volume":"101","author":[{"given":"Sandra","family":"Luikenhuis","sequence":"first","affiliation":[{"name":"Whitehead Institute for Biomedical Research, Massachusetts Institute of Technology, 9 Cambridge Center, Cambridge, MA 02142"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuela","family":"Giacometti","sequence":"additional","affiliation":[{"name":"Whitehead Institute for Biomedical Research, Massachusetts Institute of Technology, 9 Cambridge Center, Cambridge, MA 02142"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline F.","family":"Beard","sequence":"additional","affiliation":[{"name":"Whitehead Institute for Biomedical Research, Massachusetts Institute of Technology, 9 Cambridge Center, Cambridge, MA 02142"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rudolf","family":"Jaenisch","sequence":"additional","affiliation":[{"name":"Whitehead Institute for Biomedical Research, Massachusetts Institute of Technology, 9 Cambridge Center, Cambridge, MA 02142"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2004,4,6]]},"reference":[{"key":"e_1_3_2_1_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1089071"},{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddg286"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1038\/13810"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/30764"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/11.2.115"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1006\/nbdi.2001.0420"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/8.7.1253"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroscience.2003.11.025"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1044-7431(03)00026-5"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.242566899"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1086446"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1038\/85906"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1038\/85899"},{"key":"e_1_3_2_14_2","unstructured":"Sambrook J. 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