Postnatal loss of methyl-CpG binding protein 2 in the forebrain is sufficient to mediate behavioral aspects of Rett syndrome in mice

T Gemelli, O Berton, ED Nelson, LI Perrotti… - Biological …, 2006 - Elsevier
BACKGROUND: Mutations in the methyl-CpG binding protein 2 (MeCP2) gene cause Rett
syndrome (RTT), a neurodevelopmental disorder that is accompanied by a broad array of
behavioral phenotypes, mainly affecting females. Methyl-CpG binding protein 2 is a
transcriptional repressor that is widely expressed in all tissues. METHODS: To investigate
whether the postnatal loss of MeCP2 in the forebrain is sufficient to produce the behavioral
phenotypes observed in RTT, we have generated conditional MeCP2 knockout mice …