{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T09:47:06Z","timestamp":1791193626532,"version":"4.1.0"},"reference-count":48,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2014,8,5]],"date-time":"2014-08-05T00:00:00Z","timestamp":1407196800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100001819","name":"International Rett Syndrome Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100001819","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Telethon Foundation-Italy","award":["GGP09196"],"award-info":[{"award-number":["GGP09196"]}]},{"name":"Telethon Foundation-Italy","award":["GGP11095"],"award-info":[{"award-number":["GGP11095"]}]},{"name":"AIRETT-Italy"},{"name":"University of Torino-Fondazione SanPaolo"},{"DOI":"10.13039\/501100009885","name":"Regione Piemonte","doi-asserted-by":"crossref","award":["POS FESR 07\/13 BANP"],"award-info":[{"award-number":["POS FESR 07\/13 BANP"]}],"id":[{"id":"10.13039\/501100009885","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Albero di Greta ONLUS Association"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Neurochemistry"],"published-print":{"date-parts":[[2014,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Rett syndrome (RTT, <jats:styled-content style=\"fixed-case\">MIM<\/jats:styled-content>312750), a neurodevelopmental disorder predominantly occurring in females, is caused in the majority of cases by sporadic mutations in the gene encoding the transcriptional modulator methyl\u2010CpG\u2010binding protein 2 (<jats:styled-content style=\"fixed-case\">MECP<\/jats:styled-content>2). In mice, impaired Me<jats:styled-content style=\"fixed-case\">CP<\/jats:styled-content>2 function results in severe motor, cognitive, and emotional defects. The lack of <jats:italic>Mecp2<\/jats:italic> in \u03b3\u2010aminobutyric acid\u2010(GABA) releasing forebrain interneurons (INs) recapitulate many RTT features, however, the role of this gene in the development of the cortical inhibitory system is still unknown. Here, we found that Me<jats:styled-content style=\"fixed-case\">CP<\/jats:styled-content>2 expression varies among the three major classes of cortical INs and its nuclear localization differs between neuronal types. The density of calretinin<jats:sup>+<\/jats:sup> and parvalbumin<jats:sup>+<\/jats:sup> <jats:styled-content style=\"fixed-case\">IN<\/jats:styled-content>s increases in <jats:italic>Mecp2<\/jats:italic> knockout mice (<jats:italic>Mecp2<\/jats:italic><jats:sup>\u2212\/y<\/jats:sup>) already at early post\u2010natal developmental stages. In contrast, the density of somatostatin<jats:sup>+<\/jats:sup> <jats:styled-content style=\"fixed-case\">IN<\/jats:styled-content>s is not affected. We also found that the development of multipolar\u2010calretinin<jats:sup>+<\/jats:sup> <jats:styled-content style=\"fixed-case\">IN<\/jats:styled-content>s is selectively affected by the absence of <jats:italic>Mecp2<\/jats:italic>. Additionally, we show that in <jats:italic>Mecp2<\/jats:italic> heterozygous female mice, a model closely mimicking human RTT condition, <jats:styled-content style=\"fixed-case\">IN<\/jats:styled-content> abnormalities are similar to those observed in <jats:italic>Mecp2<\/jats:italic><jats:sup>\u2212\/y<\/jats:sup> mice. Together, our study indicates that loss of function of <jats:italic>Mecp2<\/jats:italic> strongly interferes with the correct establishment of the neocortical inhibitory system producing effects that are specific to different <jats:styled-content style=\"fixed-case\">IN<\/jats:styled-content> subtypes.\n<jats:boxed-text content-type=\"graphic\" position=\"anchor\"><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/jnc12803-fig-0007-m.png\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:boxed-text><\/jats:p><jats:p>The neurological consequences of the Rett syndrome\u2010related Mecp2 gene mutation are still debated. We disclosed a novel cell\u2010specific role of MeCP2 in the organization of neocortical GABAergic interneurons assembly during post\u2010natal development of the mouse brain. Parvalbumin<jats:sup>+<\/jats:sup> and calretinin<jats:sup>+<\/jats:sup> interneurons being the mostly affected. Our data suggest new avenues for the development of biomarkers and therapeutic interventions for Rett syndrome.<\/jats:p>","DOI":"10.1111\/jnc.12803","type":"journal-article","created":{"date-parts":[[2014,7,1]],"date-time":"2014-07-01T01:44:39Z","timestamp":1404179079000},"page":"115-127","source":"Crossref","is-referenced-by-count":47,"title":["Developmental abnormalities of cortical interneurons precede symptoms onset in a mouse model of Rett syndrome"],"prefix":"10.1111","volume":"131","author":[{"given":"Giulio Srubek","family":"Tomassy","sequence":"first","affiliation":[{"name":"Department of Neuroscience University of Turin Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noemi","family":"Morello","sequence":"additional","affiliation":[{"name":"Department of Neuroscience University of Turin Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleonora","family":"Calcagno","sequence":"additional","affiliation":[{"name":"Department of Neuroscience University of Turin Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurizio","family":"Giustetto","sequence":"additional","affiliation":[{"name":"Department of Neuroscience University of Turin Turin Italy"},{"name":"National Institute of Neuroscience\u2010Italy Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,8,5]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/13810"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(03)00300-3"},{"key":"e_1_2_7_4_1","first-page":"28","article-title":"Synaptic determinants of Rett syndrome","volume":"2","author":"Boggio E. 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