Cited by 1,823Genetics and Neurodevelopmental DisordersEpigenetics and DNA MethylationGenomics and Chromatin Dynamics

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MeCP2, a Key Contributor to Neurological Disease, Activates and Represses Transcription

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Chahrour, Maria, Jung, Sung Yun, Shaw, Chad, Zhou, Xiaobo, Wong, Stephen T. C., Qin, Jun, Zoghbi, Huda Y.·2008·Science

DOI 10.1126/science.1153252PMC PMC2443785

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Abstract

Mutations in the gene encoding the transcriptional repressor methyl-CpG binding protein 2 (MeCP2) cause the neurodevelopmental disorder Rett syndrome. Loss of function as well as increased dosage of the MECP2 gene cause a host of neuropsychiatric disorders. To explore the molecular mechanism(s) underlying these disorders, we examined gene expression patterns in the hypothalamus of mice that either lack or overexpress MeCP2. In both models, MeCP2 dysfunction induced changes in the expression levels of thousands of genes, but unexpectedly the majority of genes (∼85%) appeared to be activated by MeCP2. We selected six genes and confirmed that MeCP2 binds to their promoters. Furthermore, we showed that MeCP2 associates with the transcriptional activator CREB1 at the promoter of an activated target but not a repressed target. These studies suggest that MeCP2 regulates the expression of a wide range of genes in the hypothalamus and that it can function as both an activator and a repressor of transcription.

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Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2
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We thank members of the Zoghbi laboratory for their thoughtful comments Z. Yin for technical assistance M. Shinawi and A. Tawil for technical advice Y. Klisch for the art work and J. Lim J. Neul and M. Greenberg for reagents. The Baylor College of Medicine Microarray Core Facility performed the microarray experiments. This work was funded by NIH/National Institute of Neurological Disorders and Stroke grant NS057819 (H.Z.) National Institute of Child Health and Human Development Mental Retardation and Developmental Disabilities Research Center HD024064 (H.Z.) the International Rett Syndrome Foundation and the Simons Foundation. H.Z. is a Howard Hughes Medical Institute investigator. The microarray data have been deposited in the National Center for Biotechnology Information Gene Expression Omnibus (GEO) (www.ncbi.nlm.nih.gov/geo) and are accessible through GEO Series accession number GSE11150.

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Authors: Chahrour, M., Jung, S. Y., Shaw, C., Zhou, X., Wong, S. T. C., Qin, J., & Zoghbi, H. Y.
Affiliations: Baylor College of Medicine (US)
Corresponding author: Zoghbi, Huda Y.
ORCID: Chahrour, Maria
Jung, Sung Yun
Shaw, Chad
Zhou, Xiaobo
Wong, Stephen T. C.
Qin, Jun
Zoghbi, Huda Y.
Venue: Science
Publisher: American Association for the Advancement of Science (AAAS)
Published: 2008-05-30
Volume: 320
Issue: 5880
Pages: 1224-1229
ISSN: 0036-8075 (ISSN-L)
1095-9203
DOI: 10.1126/science.1153252
PMC: PMC2443785
OpenAlex: W2039276157
Cited by: 1,823
References: 22
Citations by year: 2026: 53 · 2025: 55 · 2024: 76 · 2023: 67 · 2022: 84 · 2021: 95 · 2020: 96 · 2019: 90 · 2018: 69 · 2017: 131 · 2016: 133 · 2015: 113 · 2014: 131 · 2013: 153 · 2012: 128
FWCI: 66.47
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Locations: Science (publishedVersion)
PubMed (publishedVersion)
Europe PMC (PubMed Central) (acceptedVersion)
PubMed Central (acceptedVersion)
Funders: Howard Hughes Medical Institute
National Institute of Neurological Disorders and Stroke (NS057819, R01NS057819)
Eunice Kennedy Shriver National Institute of Child Health and Human Development (HD 024064, P30HD024064)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Epigenetics and DNA Methylation
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Genomics and Chromatin Dynamics
Keywords: MeCP2, Rett syndrome, transcriptional repression, transcriptional activation, CREB1, promoter binding, hypothalamus, gene expression profiling, neuropsychiatric disorders, neurodevelopmental disorders
MeSH terms: Animals, Disease Models, Animal, Gene Expression Regulation, Hypothalamic Diseases, Hypothalamus, Male, Mice, Inbred C57BL, Nerve Tissue Proteins, Nervous System Diseases, Protein Binding, Repressor Proteins, Transcription Factors, Rett Syndrome, Transcriptional Activation, Cyclic AMP Response Element-Binding Protein, Reverse Transcriptase Polymerase Chain Reaction, Oligonucleotide Array Sequence Analysis, Mice, Methyl-CpG-Binding Protein 2

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MeCP2, a Key Contributor to Neurological Disease, Activates and Represses Transcription

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