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

The DOI matches its Crossref record; 22 of the 22 references it cites were found in a registry or an index.

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

Open access

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

Open this paper in CoChat — free account

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.

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

Integrity signals

Registry

Crossref · 10.1126/science.1153252

Cites

22 of 22 references verified0 citation errors · 0 not checkable

Statistics

Claims the paper makes

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

Self-check

Percentages, sums and reported differences re-computed from the paper’s own numbers.

The self-check needs the paper’s full text.

Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2
Amir, R. E., Van den Veyver, I. B., Wan, M., Tran, C. Q., Francke, U., & Zoghbi, H. Y. (1999) · Nature Genetics · doi:10.1038/13810 · Cited by 4,990
Self-citation
Transcriptional repression by the methyl-CpG-binding protein MeCP2 involves a histone deacetylase complex
Nan, X., Ng, H.-H., Johnson, C. A., Laherty, C. D., Turner, B. M., Eisenman, R. N., & Bird, A. (1998) · Nature · doi:10.1038/30764 · Cited by 3,440
Methylated DNA and MeCP2 recruit histone deacetylase to repress transcription
Jones, P. L., Veenstra, G. J. C., Wade, P. A., Vermaak, D., Kass, S. U., Landsberger, N., Strouboulis, J., & Wolffe, A. P. (1998) · Nature Genetics · doi:10.1038/561 · Cited by 2,791
A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome
Guy, J., Hendrich, B., Holmes, M., Martin, J. E., & Bird, A. (2001) · Nature Genetics · doi:10.1038/85899 · Cited by 1,558
DNA Methylation-Related Chromatin Remodeling in Activity-Dependent Bdnf Gene Regulation
Martinowich, K., Hattori, D., Wu, H., Fouse, S., He, F., Hu, Y., Fan, G., & Sun, Y. E. (2003) · Science · doi:10.1126/science.1090842 · Cited by 1,405
Derepression of BDNF Transcription Involves Calcium-Dependent Phosphorylation of MeCP2
Chen, W. G., Chang, Q., Lin, Y., Meissner, A., West, A. E., Griffith, E. C., Jaenisch, R., & Greenberg, M. E. (2003) · Science · doi:10.1126/science.1086446 · Cited by 1,232
Brain-Specific Phosphorylation of MeCP2 Regulates Activity-Dependent Bdnf Transcription, Dendritic Growth, and Spine Maturation
Zhou, Z., Hong, E. J., Cohen, S., Zhao, W.-N., Ho, H.-Y. H., Schmidt, L., Chen, W. G., Lin, Y., Savner, E., Griffith, E. C., et al. (2006) · Neuron · doi:10.1016/j.neuron.2006.09.037 · Cited by 877
Mild overexpression of MeCP2 causes a progressive neurological disorder in mice
Collins, A. L., Levenson, J. M., Vilaythong, A. P., Richman, R., Armstrong, D. L., Noebels, J. L., David Sweatt, J., & Zoghbi, H. Y. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddh282 · Cited by 626
Self-citation
The disease progression of Mecp2 mutant mice is affected by the level of BDNF expression
Chang, Q., Khare, G., Dani, V., Nelson, S., & Jaenisch, R. (2006) · Neuron · doi:10.1016/j.neuron.2005.12.027 · Cited by 578
MeCP2 Controls Excitatory Synaptic Strength by Regulating Glutamatergic Synapse Number
Chao, H.-T., Zoghbi, H. Y., & Rosenmund, C. (2007) · Neuron · doi:10.1016/j.neuron.2007.08.018 · Cited by 511
Self-citation

Show all 22 references

Research works printed but not linked: 3

C. L. Martinet al., Am. J. Med. Genet. B. Neuropsychiatr. Genet.144, 869 (2007).
H. Caldeira Araujoet al., Am. J. Med. Genet. A.133, 122 (2005).
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.

Sorted by citations

Items 601–700 of 999

MeCP2 controls neural stem cell fate specification through miR-199a-mediated inhibition of BMP-Smad signaling
Nakashima, H., Tsujimura, K., Irie, K., Imamura, T., Trujillo, C. A., Ishizu, M., Uesaka, M., Pan, M., Noguchi, H., Okada, K., et al. (2021) · Cell Reports · doi:10.1016/j.celrep.2021.109124 · Cited by 52
Involvement of MeCP2 in Regulation of Myelin-Related Gene Expression in Cultured Rat Oligodendrocytes
Sharma, K., Singh, J., Pillai, P. P., & Frost, E. E. (2015) · Journal of Molecular Neuroscience · doi:10.1007/s12031-015-0597-3 · Cited by 51
MicroRNAs downregulated in neuropathic pain regulate MeCP2 and BDNF related to pain sensitivity
Manners, M. T., Tian, Y., Zhou, Z., & Ajit, S. K. (2015) · FEBS Open Bio · doi:10.1016/j.fob.2015.08.010 · Cited by 50
The Chromatin-binding Protein HMGN1 Regulates the Expression of Methyl CpG-binding Protein 2 (MECP2) and Affects the Behavior of Mice
Abuhatzira, L., Shamir, A., Schones, D. E., Schäffer, A. A., & Bustin, M. (2011) · Journal of Biological Chemistry · doi:10.1074/jbc.m111.300541 · Cited by 53
Aberrant redox homoeostasis and mitochondrial dysfunction in Rett syndrome
Müller, M., & Can, K. (2014) · Biochemical Society Transactions · doi:10.1042/bst20140071 · Cited by 50
Epigenetics DNA methylation in the core ataxin-2 gene promoter: novel physiological and pathological implications
Laffita‐Mesa, J. M., Bauer, P. O., Kourí, V., Serrano, L. P., Roskams, J., Gotay, D. A., Brown, J. C. M., Martínez, P. A., González-Zaldívar, Y., Almaguer-Mederos, L. E., et al. (2011) · Human Genetics · doi:10.1007/s00439-011-1101-y · Cited by 52
Persistent Pain Maintains Morphine-Seeking Behavior after Morphine Withdrawal through Reduced MeCP2 Repression of Glua1 in Rat Central Amygdala
Hou, Y.-Y., Cai, Y.-Q., & Pan, Z. Z. (2015) · Journal of Neuroscience · doi:10.1523/jneurosci.3453-14.2015 · Cited by 51
Inherited human IRAK-1 deficiency selectively impairs TLR signaling in fibroblasts
Mina, E. D., Borghesi, A., Zhou, H., Bougarn, S., Boughorbel, S., Israël, L., Meloni, I., Chrabieh, M., Ling, Y., Itan, Y., et al. (2017) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1620139114 · Cited by 47
Lack of IL-1R8 in neurons causes hyperactivation of IL-1 receptor pathway and induces MECP2-dependent synaptic defects
Tomasoni, R., Morini, R., López‐Atalaya, J. P., Corradini, I., Canzi, A., Rasile, M., Mantovani, C., Pozzi, D., Garlanda, C., Mantovani, A., et al. (2017) · eLife · doi:10.7554/elife.21735 · Cited by 52
Sex differences in Mecp2-mutant Rett syndrome model mice and the impact of cellular mosaicism in phenotype development
Ribeiro, M. C., & MacDonald, J. L. (2020) · Brain Research · doi:10.1016/j.brainres.2019.146644 · Cited by 55
Drug Studies on Rett Syndrome: From Bench to Bedside
Mohan, G., Padmapriya, S., & Vellingiri, B. (2020) · Journal of Autism and Developmental Disorders · doi:10.1007/s10803-020-04381-y · Cited by 53
The role and molecular mechanism of epigenetics in cardiac hypertrophy
Lei, H. C., Hu, J., Sun, K., & Xu, D. (2020) · Heart Failure Reviews · doi:10.1007/s10741-020-09959-3 · Cited by 51
Epigenetic Mechanisms in Autism Spectrum Disorder
Zhubi, A., Cook, E. H., Guidotti, A., & Grayson, D. R. (2014) · International review of neurobiology · doi:10.1016/b978-0-12-801311-3.00006-8 · Cited by 56
MeCP2: multifaceted roles in gene regulation and neural development
Cheng, T., & Qiu, Z. (2014) · Neuroscience Bulletin · doi:10.1007/s12264-014-1452-6 · Cited by 55
MeCP2-Related Diseases and Animal Models
Ezeonwuka, C. D., & Rastegar, M. (2014) · Diseases · doi:10.3390/diseases2010045 · Cited by 49
Targeting Androgen Receptor Leads to Suppression of Prostate Cancer via Induction of Autophagy
Jiang, Q., Yeh, S., Wang, X., Xu, D., Zhang, Q., Wen, X., Xia, S., & Chang, C. (2012) · The Journal of Urology · doi:10.1016/j.juro.2012.06.004 · Cited by 50
Merging Data from Genetic and Epigenetic Approaches to Better Understand Autistic Spectrum Disorder
Grayson, D. R., & Guidotti, A. (2015) · Epigenomics · doi:10.2217/epi.15.92 · Cited by 51
Induced pluripotent stem cells in the study of neurological diseases
Saporta, M., Grs̆ković, M., & Dimos, J. T. (2011) · Stem Cell Research & Therapy · doi:10.1186/scrt78 · Cited by 50
MeCP2 and Chromatin Compartmentalization
Schmidt, A., Zhang, H., & Cardoso, M. C. (2020) · Cells · doi:10.3390/cells9040878 · Cited by 51
Caregiver maltreatment causes altered neuronal DNA methylation in female rodents
Blaze, J., & Roth, T. L. (2017) · Development and Psychopathology · doi:10.1017/s0954579417000128 · Cited by 48
Role of Mecp2 in Experience-Dependent Epigenetic Programming
Zimmermann, C. A., Hoffmann, A., Raabe, F. J., & Spengler, D. (2015) · Genes · doi:10.3390/genes6010060 · Cited by 51
Early Postnatal Nutrition Determines Adult Physical Activity and Energy Expenditure in Female Mice
Li, G., Kohorst, J. J., Zhang, W., Laritsky, E., Kunde‐Ramamoorthy, G., Baker, M. S., Fiorotto, M. L., & Waterland, R. A. (2013) · Diabetes · doi:10.2337/db12-1306 · Cited by 49
Mecp2 regulates neural cell differentiation by suppressing the Id1 to Her2 axis in zebrafish
Gao, H. F., Bu, Y., Wu, Q., Wang, X., Chang, N., Lei, L., Chen, S., Liu, D. N., Zhu, X., Hu, K., et al. (2015) · Journal of Cell Science · doi:10.1242/jcs.167874 · Cited by 48
From Memory Impairment to Posttraumatic Stress Disorder-Like Phenotypes: The Critical Role of an Unpredictable Second Traumatic Experience
Finsterwald, C., Steinmetz, A. B., Travaglia, A., & Alberini, C. M. (2015) · Journal of Neuroscience · doi:10.1523/jneurosci.0771-15.2015 · Cited by 50
MeCP2 and the enigmatic organization of brain chromatin. Implications for depression and cocaine addiction
Ausió, J. (2016) · Clinical Epigenetics · doi:10.1186/s13148-016-0214-5 · Cited by 49
Increased Mitochondrial Mass and Cytosolic Redox Imbalance in Hippocampal Astrocytes of a Mouse Model of Rett Syndrome: Subcellular Changes Revealed by Ratiometric Imaging of JC‐1 and roGFP1 Fluorescence
Bebensee, D. F., Can, K., & Müller, M. (2017) · Oxidative Medicine and Cellular Longevity · doi:10.1155/2017/3064016 · Cited by 49
Activity-dependent aberrations in gene expression and alternative splicing in a mouse model of Rett syndrome
Osenberg, S., Karten, A., Sun, J., Li, J., Charkowick, S. V., Felice, C. A., Kritzer, M. F., Nguyen, M. V. C., Yu, P., & Ballas, N. (2018) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1722546115 · Cited by 46
Redefining transcriptional regulation of the APOE gene and its association with Alzheimer’s disease
Lee, E.-G., Tulloch, J., Chen, S., Leong, L., Saxton, A. D., Kraemer, B. C., Darvas, M., Keene, C. D., Shutes‐David, A., Todd, K. W., et al. (2020) · PLoS ONE · doi:10.1371/journal.pone.0227667 · Cited by 49
IUGR differentially alters MeCP2 expression and H3K9Me3 of the PPARγ gene in male and female rat lungs during alveolarization
Joss‐Moore, L. A., Wang, Y., Ogata, E. M., Sainz, A., Yu, X., Callaway, C. W., McKnight, R. A., Albertine, K. H., & Lane, R. H. (2011) · Birth Defects Research Part A Clinical and Molecular Teratology · doi:10.1002/bdra.20783 · Cited by 48
Krüppel-homolog 1 exerts anti-metamorphic and vitellogenic functions in insects via phosphorylation-mediated recruitment of specific cofactors
Wu, Z., Yang, L., Li, H., & Zhou, S. (2021) · BMC Biology · doi:10.1186/s12915-021-01157-3 · Cited by 54
Molecular and synaptic defects in intellectual disability syndromes
Verpelli, C., & Sala, C. (2011) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2011.09.007 · Cited by 55
Elevated Transcription Factor Specificity Protein 1 in Autistic Brains Alters the Expression of Autism Candidate Genes
Thanseem, I., Anitha, A., Nakamura, K., Suda, S., Iwata, K., Matsuzaki, H., Ohtsubo, M., Ueki, T., Katayama, T., Iwata, Y., et al. (2011) · Biological Psychiatry · doi:10.1016/j.biopsych.2011.09.020 · Cited by 49
DNA Modifications and Neurological Disorders
Weng, Y., An, R., Shin, J., Song, H., & Ming, G. (2013) · Neurotherapeutics · doi:10.1007/s13311-013-0223-4 · Cited by 49
One-carbon metabolism in neurodevelopmental disorders: Using broad-based nutraceutics to treat cognitive deficits in complex spectrum disorders
Schaevitz, L. R., Berger-Sweeney, J. E., & Ricceri, L. (2014) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2014.04.007 · Cited by 50
MeCP2 as an Activator of Gene Expression
Horvath, P. M., & Monteggia, L. M. (2018) · Trends in Neurosciences · doi:10.1016/j.tins.2017.11.005 · Cited by 47
Transcriptional and splicing dysregulation in the prefrontal cortex in valproic acid rat model of autism
Zhang, R., Zhou, J., Ren, J., Sun, S., Di, Y., Wang, H., An, X., Zhang, K., Zhang, J., Qian, Z., et al. (2018) · Reproductive Toxicology · doi:10.1016/j.reprotox.2018.01.008 · Cited by 47
Role of MeCP2, DNA methylation, and HDACs in regulating synapse function
Kavalali, E. T., Nelson, E. D., & Monteggia, L. M. (2011) · Journal of Neurodevelopmental Disorders · doi:10.1007/s11689-011-9078-3 · Cited by 46
Dual-role transcription factors stabilize intermediate expression levels
He, J., Huo, X., Pei, G., Jia, Z., Yan, Y., Yu, J., Qu, H., Xie, Y., Yuan, J., Zheng, Y., et al. (2024) · Cell · doi:10.1016/j.cell.2024.03.023 · Cited by 46
Discovery of Rare Mutations in Autism: Elucidating Neurodevelopmental Mechanisms
Uzun, E. D. G., Sciarra, L. N., Maguire, A. M., Pescosolido, M. F., van Dyck, L. I., & Morrow, E. M. (2015) · Neurotherapeutics · doi:10.1007/s13311-015-0363-9 · Cited by 45
Rett syndrome treatment in mouse models: Searching for effective targets and strategies
Ricceri, L., Filippis, B. D., & Laviola, G. (2012) · Neuropharmacology · doi:10.1016/j.neuropharm.2012.08.010 · Cited by 46
Adult reversal of cognitive phenotypes in neurodevelopmental disorders
Silva, A. J., & Ehninger, D. (2009) · Journal of Neurodevelopmental Disorders · doi:10.1007/s11689-009-9018-7 · Cited by 45
Insulinotropic treatments exacerbate metabolic syndrome in mice lacking MeCP2 function
Pitcher, M. R., Ward, C. S., Arvide, E. M., Chapleau, C. A., Pozzo‐Miller, L. D., Hoeflich, A., Sivaramakrishnan, M., Saenger, S., Metzger, F., & Neul, J. L. (2013) · Human Molecular Genetics · doi:10.1093/hmg/ddt111 · Cited by 47
The Epstein–Barr virus nuclear antigen-1 reprograms transcription by mimicry of high mobility group A proteins
Coppotelli, G., Mughal, N., Callegari, S., Sompallae, R. R., Caja, L., Luijsterburg, M. S., Dantuma, N. P., Moustakas, A., & Masucci, M. G. (2013) · Nucleic Acids Research · doi:10.1093/nar/gkt032 · Cited by 46
Tomato MBD 5, a methyl CpG binding domain protein, physically interacting with UV ‐damaged DNA binding protein‐1, functions in multiple processes
Li, Y., Deng, H., Miao, M., Li, H., Huang, S., Wang, S., & Liu, Y. (2015) · New Phytologist · doi:10.1111/nph.13745 · Cited by 46
Scale Invariant Disordered Nanotopography Promotes Hippocampal Neuron Development and Maturation with Involvement of Mechanotransductive Pathways
Schulte, C., Ripamonti, M., Maffioli, E., Cappelluti, M. A., Nonnis, S., Puricelli, L., Lamanna, J., Piazzoni, C., Podestà, A., Lenardi, C., et al. (2016) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2016.00267 · Cited by 44
Total RNA Sequencing of Rett Syndrome Autopsy Samples Identifies the M4 Muscarinic Receptor as a Novel Therapeutic Target
Gogliotti, R. G., Fisher, N. M., Stansley, B. J., Jones, C. K., Lindsley, C. W., Conn, P. J., & Niswender, C. M. (2018) · Journal of Pharmacology and Experimental Therapeutics · doi:10.1124/jpet.117.246991 · Cited by 45
Disturbed redox homeostasis and oxidative stress: Potential players in the developmental regression in Rett syndrome
Müller, M. (2019) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2018.12.009 · Cited by 47
Accelerated Hyper-Maturation of Parvalbumin Circuits in the Absence of MeCP2
Patrizi, A., Awad, P. N., Chattopadhyaya, B., Li, C., Di Cristo, G., & Fagiolini, M. (2020) · Cerebral Cortex · doi:10.1093/cercor/bhz085 · Cited by 46
MECP2 mutations affect ciliogenesis: a novel perspective for Rett syndrome and related disorders
Frasca, A., Spiombi, E., Palmieri, M., Albizzati, E., Valente, M. M., Bergo, A., Leva, B., Kilstrup‐Nielsen, C., Bianchi, F., Di Carlo, V., et al. (2020) · EMBO Molecular Medicine · doi:10.15252/emmm.201910270 · Cited by 46
Dental Pulp-Derived Mesenchymal Stem Cells for Modeling Genetic Disorders
Masuda, K., Han, X., Kato, H., Sato, H., Zhang, Y., Sun, X., Hirofuji, Y., Yamaza, H., Yamada, A., & Fukumoto, S. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22052269 · Cited by 49
Gender influences monoallelic expression of ATP10A in human brain
Hogart, A., Patzel, K. A., & LaSalle, J. M. (2008) · Human Genetics · doi:10.1007/s00439-008-0546-0 · Cited by 46
Activating a Repressor
Cohen, S., Zhou, Z., & Greenberg, M. E. (2008) · Science · doi:10.1126/science.1159146 · Cited by 48
MeCP2-induced heterochromatin organization is driven by oligomerization-based liquid–liquid phase separation and restricted by DNA methylation
Zhang, H., Romero, H., Schmidt, A., Gagova, K., Qin, W., Bertulat, B., Lehmkuhl, A., Milden, M., Eck, M., Meckel, T., et al. (2022) · Nucleus · doi:10.1080/19491034.2021.2024691 · Cited by 44
Differential expression of m5C RNA methyltransferase genes NSUN6 and NSUN7 in Alzheimer’s disease and traumatic brain injury
PerezGrovas‐Saltijeral, A., Rajkumar, A. P., & Knight, H. M. (2023) · Molecular Neurobiology · doi:10.1007/s12035-022-03195-6 · Cited by 46
MeCP2 Regulates the Synaptic Expression of a Dysbindin-BLOC-1 Network Component in Mouse Brain and Human Induced Pluripotent Stem Cell-Derived Neurons
Larimore, J. L., Ryder, P. V., Kim, K., Ambrose, L. A., Chapleau, C. A., Calfa, G. D., Groß, C., Bassell, G. J., Pozzo‐Miller, L. D., Smith, Y., et al. (2013) · PLoS ONE · doi:10.1371/journal.pone.0065069 · Cited by 45
Mild Overexpression of Mecp2 in Mice Causes a Higher Susceptibility toward Seizures
Bodda, C., Tantra, M., Mollajew, R., Arunachalam, J. P., Laccone, F. A., Can, K., Rosenberger, A., Mironov, S. L., Ehrenreich, H., & Mannan, A. U. (2013) · American Journal Of Pathology · doi:10.1016/j.ajpath.2013.03.019 · Cited by 44
Prenatal immune activation alters the adult neural epigenome but can be partly stabilised by a n-3 polyunsaturated fatty acid diet
Basil, P., Li, Q., Gui, H., Hui, T. C. K., Ling, V. H. M., Wong, C. C. Y., Mill, J. S., McAlonan, G. M., & Sham, P. C. (2018) · Translational Psychiatry · doi:10.1038/s41398-018-0167-x · Cited by 47
Chromatin-dependent allosteric regulation of DNMT3A activity by MeCP2
Rajavelu, A., Lungu, C., Emperle, M., Dukatz, M., Bröhm, A., Broche, J., Hanelt, I., Parsa, E., Schiffers, S., Karnik, R., et al. (2018) · Nucleic Acids Research · doi:10.1093/nar/gky715 · Cited by 48
Laser capture microdissection–reduced representation bisulfite sequencing (LCM-RRBS) maps changes in DNA methylation associated with gonadectomy-induced adrenocortical neoplasia in the mouse
Schillebeeckx, M., Schrade, A., Löbs, A., Pihlajoki, M., Wilson, D. B., & Mitra, R. D. (2013) · Nucleic Acids Research · doi:10.1093/nar/gkt230 · Cited by 44
In vitro neurogenesis: development and functional implications of iPSC technology
Compagnucci, C., Nizzardo, M., Corti, S. P., Zanni, G., & Bertini, E. (2013) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-013-1511-1 · Cited by 43
Subclinical myocardial dysfunction in Rett syndrome
Felice, C. D., Maffei, S., Signorini, C., Leoncini, S., Lunghetti, S., Valacchi, G., D’Esposito, M., Filosa, S., Ragione, F. D., Butera, G., et al. (2011) · European Heart Journal - Cardiovascular Imaging · doi:10.1093/ejechocard/jer256 · Cited by 44
Nanoscale bio-platforms for living cell interrogation: current status and future perspectives
Chang, L., Hu, J., Chen, F., Chen, Z., Shi, J., Yang, Z., Li, Y., & Lee, L. J. (2015) · Nanoscale · doi:10.1039/c5nr06694h · Cited by 44
Epigenetic marks and their relationship with BDNF in the brain of suicide victims
Misztak, P., Pańczyszyn-Trzewik, P., Nowak, G., & Sowa-Kućma, M. (2020) · PLoS ONE · doi:10.1371/journal.pone.0239335 · Cited by 44
DNA methylation signatures of autoimmune diseases in human B lymphocytes
Zouali, M. (2020) · Clinical Immunology · doi:10.1016/j.clim.2020.108622 · Cited by 45
The Molecular Functions of MeCP2 in Rett Syndrome Pathology
Sharifi, O., & Yasui, D. H. (2021) · Frontiers in Genetics · doi:10.3389/fgene.2021.624290 · Cited by 49
Neonatal MeCP2 is important for the organization of sex differences in vasopressin expression
Forbes-Lorman, R. M., Rautio, J. J., Kurian, J. R., Auger, A. P., & Auger, C. J. (2012) · Epigenetics · doi:10.4161/epi.7.3.19265 · Cited by 43
From Basic Visual Science to Neurodevelopmental Disorders: The Voyage of Environmental Enrichment-Like Stimulation
Consorti, A., Sansevero, G., Torelli, C., Berardi, N., & Sale, A. (2019) · Neural Plasticity · doi:10.1155/2019/5653180 · Cited by 44
State‐of‐the‐art therapies for Rett syndrome
Panayotis, N., Ehinger, Y., Félix, M., & Roux, J. (2022) · Developmental Medicine & Child Neurology · doi:10.1111/dmcn.15383 · Cited by 46
MeCP2 Lactylation Protects against Ischemic Brain Injury by Transcriptionally Regulating Neuronal Apoptosis
Sun, M., Zhang, Y., Mao, R., Chen, Y., Liu, P., Ye, L., Xu, S., Jia, J., Shu, S., Li, H., et al. (2025) · Advanced Science · doi:10.1002/advs.202415309 · Cited by 44
Kaiso is a bimodal modulator for Wnt/β‐catenin signaling
Iioka, H., Doerner, S. K., & Tamai, K. (2009) · FEBS Letters · doi:10.1016/j.febslet.2009.01.012 · Cited by 42
Targeting epigenetic mechanisms for pain relief
Géranton, S. M. (2011) · Current Opinion in Pharmacology · doi:10.1016/j.coph.2011.10.012 · Cited by 45
Epigenetic regulation of self‐renewal and fate determination in neural stem cells
Ariff, I. M., Mitra, A., & Basu, A. (2011) · Journal of Neuroscience Research · doi:10.1002/jnr.22804 · Cited by 48
Epileptogenesis: Can the Science of Epigenetics Give Us Answers?
Lubin, F. D. (2012) · Epiliepsy currents/Epilepsy currents · doi:10.5698/1535-7511-12.3.105 · Cited by 43
CpG Methylation in Neurons: Message, Memory, or Mask?
Sharma, R. P., Gavin, D. P., & Grayson, D. R. (2010) · Neuropsychopharmacology · doi:10.1038/npp.2010.85 · Cited by 43
Negative Allosteric Modulation of mGluR5 Partially Corrects Pathophysiology in a Mouse Model of Rett Syndrome
Tao, J., Wu, H., Coronado, A. A., de Laittre, E. A., Osterweil, E. K., Zhang, Y., & Bear, M. F. (2016) · Journal of Neuroscience · doi:10.1523/jneurosci.0672-16.2016 · Cited by 46
Somatostatin-IRES-Cre Mice: Between Knockout and Wild-Type?
Viollet, C., Simon, A., Tolle, V., Labarthe, A., Grouselle, D., Loe-Mie, Y., Simonneau, M., Martel, G., & Epelbaum, J. (2017) · Frontiers in Endocrinology · doi:10.3389/fendo.2017.00131 · Cited by 48
Fibrogenic Activity of MECP2 Is Regulated by Phosphorylation in Hepatic Stellate Cells
Morán‐Salvador, E., Macia, M. G., Sivaharan, A., Sabater, L., Zaki, M. Y. W., Oakley, F., Knox, A., Page, A., Luli, S., Mann, J., et al. (2019) · Gastroenterology · doi:10.1053/j.gastro.2019.07.029 · Cited by 43
Epigenetic Mechanisms of Learning and Memory: Implications for Aging
Creighton, S. D., Stefanelli, G., Reda, A. J., & Zovkic, I. B. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21186918 · Cited by 45
Clustered Protocadherins Emerge as Novel Susceptibility Loci for Mental Disorders
Jia, Z., & Wu, Q. (2020) · Frontiers in Neuroscience · doi:10.3389/fnins.2020.587819 · Cited by 41
Disruption of MeCP2–TCF20 complex underlies distinct neurodevelopmental disorders
Zhou, J., Hamdan, H., Yalamanchili, H. K., Pang, K., Pohodich, A. E., Lopez, J., Shao, Y., Osés-Prieto, J. A., Li, L., Kim, W., et al. (2022) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2119078119 · Cited by 44
Self-citation
CX3CR1 ablation ameliorates motor and respiratory dysfunctions and improves survival of a Rett syndrome mouse model
Horiuchi, M., Smith, L., Maezawa, I., & Jin, L. (2016) · Brain Behavior and Immunity · doi:10.1016/j.bbi.2016.02.014 · Cited by 42
Transcription factors: Time to deliver
Ulasov, A. V., Rosenkranz, A. A., & Sobolev, A. S. (2017) · Journal of Controlled Release · doi:10.1016/j.jconrel.2017.11.004 · Cited by 44
Apparent bias toward long gene misregulation in MeCP2 syndromes disappears after controlling for baseline variations
Raman, A. T., Pohodich, A. E., Wan, Y., Yalamanchili, H. K., Lowry, W. E., Zoghbi, H. Y., & Liu, Z. (2018) · Nature Communications · doi:10.1038/s41467-018-05627-1 · Cited by 42
Self-citation
MeCP2 nuclear dynamics in live neurons results from low and high affinity chromatin interactions
Piccolo, F. M., Liu, Z. J., Dong, P., Hsu, C.-L., Stoyanova, E. I., Rao, A., Tjian, R., & Heintz, N. (2019) · eLife · doi:10.2139/ssrn.3376659 · Cited by 38
Ion Channel Mutations in Neuronal Diseases: A Genetics Perspective
Steinlein, O. K. (2012) · Chemical Reviews · doi:10.1021/cr300044d · Cited by 43
Gene expression of epigenetic regulatory factors related to primary silencing mechanism is less susceptible to lower doses of bisphenol A in embryonic hypothalamic cells
Warita, K., Mitsuhashi, T., Ohta, K.-I., Suzuki, S., Hoshi, N., Miki, T., & Takeuchi, Y. (2013) · The Journal of Toxicological Sciences · doi:10.2131/jts.38.285 · Cited by 41
Primary Cilia Dysfunction in Neurodevelopmental Disorders beyond Ciliopathies
Karalis, V., Donovan, K. E., & Şahin, M. (2022) · Journal of Developmental Biology · doi:10.3390/jdb10040054 · Cited by 39
Sex Differences in Brain Disorders
Ziemka‐Nałęcz, M., Pawelec, P., Ziąbska, K., & Zalewska, T. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms241914571 · Cited by 48
Synaptic plasticity and signaling in rett syndrome
Sala, G. D., & Pizzorusso, T. (2013) · Developmental Neurobiology · doi:10.1002/dneu.22114 · Cited by 42
Knock-down of methyl CpG-binding protein 2 (MeCP2) causes alterations in cell proliferation and nuclear lamins expression in mammalian cells
Babbio, F., Castiglioni, I., Cassina, C., Gariboldi, M. B., Pistore, C., Magnani, E., Badaracco, G., Monti, E., & Bonapace, I. M. (2012) · BMC Cell Biology · doi:10.1186/1471-2121-13-19 · Cited by 40
iPSCs and small molecules: a reciprocal effort towards better approaches for drug discovery
Zhang, R., Zhang, L., & Xie, X. (2013) · Acta Pharmacologica Sinica · doi:10.1038/aps.2013.21 · Cited by 42
Astrocytes conspire with neurons during progression of neurological disease
McGann, J. C., Lioy, D. T., & Mandel, G. (2012) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2012.03.009 · Cited by 43
Heat stress enhances LTM formation inLymnaea: role of HSPs and DNA methylation
Sunada, H., Riaz, H., de Freitas, E., Lukowiak, K. S., Swinton, E., Swinton, E., Protheroe, A., Shymansky, T., Komatsuzaki, Y., & Lukowiak, K. D. (2016) · Journal of Experimental Biology · doi:10.1242/jeb.134296 · Cited by 41
MECP2 and the biology of MECP2 duplication syndrome
D’Mello, S. R. (2021) · Journal of Neurochemistry · doi:10.1111/jnc.15331 · Cited by 42
The role of GABAergic system in neurodevelopmental disorders: a focus on autism and epilepsy.
Sgadó, P., Dunleavy, M., Genovesi, S., Provenzano, G., & Bozzi, Y. (2011) · PubMed Central · Cited by 40
MeCP2 and histone deacetylases 1 and 2 in dorsal striatum collectively suppress repetitive behaviors
Mahgoub, M., Adachi, M., Suzuki, K., Liu, X., Kavalali, E. T., Chahrour, M. H., & Monteggia, L. M. (2016) · Nature Neuroscience · doi:10.1038/nn.4395 · Cited by 40
Self-citation
Activity-Dependent Plasticity and Gene Expression Modifications in the Adult CNS
Carulli, D., Foscarin, S., & Rossi, F. (2011) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2011.00050 · Cited by 38
Disruption of Me CP 2 attenuates circadian rhythm in CRISPR /Cas9‐based Rett syndrome model mouse
Tsuchiya, Y., Minami, Y., Umemura, Y. M., Watanabe, H., Ono, D., Nakamura, W., Takahashi, T., Honma, S., Kondoh, G., Matsuishi, T., et al. (2015) · Genes to Cells · doi:10.1111/gtc.12305 · Cited by 41
Biological embedding in mental health: An epigenomic perspective
Sasaki, A., de Vega, W. C., & McGowan, P. O. (2013) · Biochemistry and Cell Biology · doi:10.1139/bcb-2012-0070 · Cited by 43
MicroRNAs in Opioid Pharmacology
Hwang, C. K., Wagley, Y., Law, P., Wei, L., & Loh, H. H. (2011) · Journal of Neuroimmune Pharmacology · doi:10.1007/s11481-011-9323-2 · Cited by 41

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

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review

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

Abstract

Overview

Integrity signals

Verification

Claims the paper makes

Claims other papers make about this one

Self-check

Citations

Cites

Cited by

Related

Metadata

Record

About these checks