PMID- 17278130 OWN - NLM STAT- MEDLINE DCOM- 20070501 LR - 20161122 IS - 0021-9967 (Print) IS - 0021-9967 (Linking) VI - 501 IP - 4 DP - 2007 Apr 1 TI - Differential distribution of the MeCP2 splice variants in the postnatal mouse brain. PG - 526-42 AB - Mutations in the gene encoding methyl CpG binding protein 2 (MeCP2) are the primary cause of the neurodevelopmental disorder Rett syndrome (RTT). Mecp2-deficient mice develop a neurological phenotype that recapitulates many of the symptoms of RTT, including postnatal onset of the neurological deficits. MeCP2 has two isoforms, MeCP2e1 and MeCP2e2, with distinct amino termini, which are generated by alternative splicing. We examined the distribution of the Mecp2 splice variants in the postnatal mouse brain by in situ hybridization and found regional and age-related differences in transcript abundance. In newborn mice, signals for total Mecp2 and the Mecp2e2 transcripts were widely distributed, with overlapping expression patterns throughout the brain. Expression of the Mecp2e2 splice variant became largely restricted to nuclei within the dorsal thalamus (DT) and cortical layer V in juvenile animals, a pattern that was maintained into adulthood. In contrast, the total Mecp2 riboprobe only weakly labeled the DT and cortical layer V in juvenile and adult animals, although it heavily labeled surrounding brain regions, suggesting that Mecp2e1 is the predominant transcript outside the thalamus. Quantitative real-time PCR was used to measure Mecp2e1 and Mecp2e2 abundance in the diencephalon of adult mice, demonstrating significantly more Mecp2e2 in the DT than in the hypothalamus, which is in agreement with the Mecp2e2 in situ hybridization. The differential distribution of the Mecp2e1 and Mecp2e2 transcripts indicates regional and developmental regulation of Mecp2 splicing in the postnatal mouse brain. CI - (c) 2007 Wiley-Liss, Inc. FAU - Dragich, Joanna M AU - Dragich JM AD - Neuroscience Interdepartmental PhD Program, University of California, Los Angeles, Los Angeles, California 90095-1761, USA. FAU - Kim, Yong-Hwan AU - Kim YH FAU - Arnold, Arthur P AU - Arnold AP FAU - Schanen, N Carolyn AU - Schanen NC LA - eng GR - F31 NS043072/NS/NINDS NIH HHS/United States GR - F31 NS043072-03/NS/NINDS NIH HHS/United States GR - R01HD37874/HD/NICHD NIH HHS/United States GR - P20RR020173/RR/NCRR NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - J Comp Neurol JT - The Journal of comparative neurology JID - 0406041 RN - 0 (Mecp2 protein, mouse) RN - 0 (Methyl-CpG-Binding Protein 2) RN - 0 (RNA, Messenger) SB - IM MH - Age Factors MH - *Alternative Splicing MH - Animals MH - Animals, Newborn MH - Brain/growth & development/*metabolism MH - Brain Mapping MH - Female MH - Gene Expression Regulation, Developmental/*physiology MH - In Situ Hybridization/methods MH - Male MH - Methyl-CpG-Binding Protein 2/*genetics MH - Mice MH - Mice, Inbred C57BL MH - RNA, Messenger MH - Reverse Transcriptase Polymerase Chain Reaction/methods EDAT- 2007/02/06 09:00 MHDA- 2007/05/02 09:00 CRDT- 2007/02/06 09:00 PHST- 2007/02/06 09:00 [pubmed] PHST- 2007/05/02 09:00 [medline] PHST- 2007/02/06 09:00 [entrez] AID - 10.1002/cne.21264 [doi] PST - ppublish SO - J Comp Neurol. 2007 Apr 1;501(4):526-42. doi: 10.1002/cne.21264.