Reversal of neurological defects in a mouse model of Rett syndrome.

A model for neural development and treatment of Rett syndrome using human induced pluripotent stem cells. Cell (2010) 7.51

Neuronal MeCP2 is expressed at near histone-octamer levels and globally alters the chromatin state. Mol Cell (2010) 5.43

Behavioural phenotyping assays for mouse models of autism. Nat Rev Neurosci (2010) 5.27

Signaling mechanisms linking neuronal activity to gene expression and plasticity of the nervous system. Annu Rev Neurosci (2008) 5.12

CpG islands influence chromatin structure via the CpG-binding protein Cfp1. Nature (2010) 4.86

Dendritic spine pathology in neuropsychiatric disorders. Nat Neurosci (2011) 4.80

Absence of CNTNAP2 leads to epilepsy, neuronal migration abnormalities, and core autism-related deficits. Cell (2011) 4.58

MeCP2 controls excitatory synaptic strength by regulating glutamatergic synapse number. Neuron (2007) 4.15

Integrated epigenomic analyses of neuronal MeCP2 reveal a role for long-range interaction with active genes. Proc Natl Acad Sci U S A (2007) 4.00

Wild-type microglia arrest pathology in a mouse model of Rett syndrome. Nature (2012) 3.83

Reduced social interaction and ultrasonic communication in a mouse model of monogenic heritable autism. Proc Natl Acad Sci U S A (2008) 3.74

Autism. Lancet (2009) 3.68

Non-cell autonomous influence of MeCP2-deficient glia on neuronal dendritic morphology. Nat Neurosci (2009) 3.42

L1 retrotransposition in neurons is modulated by MeCP2. Nature (2010) 3.25

Partial reversal of Rett Syndrome-like symptoms in MeCP2 mutant mice. Proc Natl Acad Sci U S A (2009) 3.23

A role for glia in the progression of Rett's syndrome. Nature (2011) 3.18

Interneuron dysfunction in psychiatric disorders. Nat Rev Neurosci (2012) 2.87

Advances in autism. Annu Rev Med (2009) 2.75

Negative allosteric modulation of the mGluR5 receptor reduces repetitive behaviors and rescues social deficits in mouse models of autism. Sci Transl Med (2012) 2.70

The conundrums of understanding genetic risks for autism spectrum disorders. Nat Neurosci (2011) 2.61

A suppressor screen in Mecp2 mutant mice implicates cholesterol metabolism in Rett syndrome. Nat Genet (2013) 2.59

Synaptic dysfunction in neurodevelopmental disorders associated with autism and intellectual disabilities. Cold Spring Harb Perspect Biol (2012) 2.42

Communication between the synapse and the nucleus in neuronal development, plasticity, and disease. Annu Rev Cell Dev Biol (2008) 2.40

Rett syndrome astrocytes are abnormal and spread MeCP2 deficiency through gap junctions. J Neurosci (2009) 2.35

Phosphorylation of MeCP2 at Serine 80 regulates its chromatin association and neurological function. Proc Natl Acad Sci U S A (2009) 2.30

Astrocytes and disease: a neurodevelopmental perspective. Genes Dev (2012) 2.28

Activity-dependent neuronal signalling and autism spectrum disorder. Nature (2013) 2.23

Forniceal deep brain stimulation rescues hippocampal memory in Rett syndrome mice. Nature (2015) 2.18

Balanced gene regulation by an embryonic brain ncRNA is critical for adult hippocampal GABA circuitry. Nat Neurosci (2009) 2.15

GABAergic Neuron-Specific Loss of Ube3a Causes Angelman Syndrome-Like EEG Abnormalities and Enhances Seizure Susceptibility. Neuron (2016) 2.14

DNA methylation and methyl-CpG binding proteins: developmental requirements and function. Chromosoma (2009) 2.13

Repetitive self-grooming behavior in the BTBR mouse model of autism is blocked by the mGluR5 antagonist MPEP. Neuropsychopharmacology (2009) 2.10

Adult neural function requires MeCP2. Science (2011) 2.09

Autism and brain development. Cell (2008) 2.01

Common variants within MECP2 confer risk of systemic lupus erythematosus. PLoS One (2008) 1.98

Toward fulfilling the promise of molecular medicine in fragile X syndrome. Annu Rev Med (2011) 1.96

Molecular neuroanatomy's "Three Gs": a primer. Neuron (2007) 1.91

Global transcriptional and translational repression in human-embryonic-stem-cell-derived Rett syndrome neurons. Cell Stem Cell (2013) 1.80

Reversing neurodevelopmental disorders in adults. Neuron (2008) 1.80

Experience-dependent epigenetic modifications in the central nervous system. Biol Psychiatry (2008) 1.79

DNA methylation and its basic function. Neuropsychopharmacology (2012) 1.76

Rett syndrome mutations abolish the interaction of MeCP2 with the NCoR/SMRT co-repressor. Nat Neurosci (2013) 1.74

Preclinical research in Rett syndrome: setting the foundation for translational success. Dis Model Mech (2012) 1.66

MeCP2 binds to non-CG methylated DNA as neurons mature, influencing transcription and the timing of onset for Rett syndrome. Proc Natl Acad Sci U S A (2015) 1.63

Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy. J Clin Invest (2011) 1.62

Peripheral Mechanosensory Neuron Dysfunction Underlies Tactile and Behavioral Deficits in Mouse Models of ASDs. Cell (2016) 1.58

DNA methylation in mammals. Cold Spring Harb Perspect Biol (2014) 1.57

Synaptic circuit abnormalities of motor-frontal layer 2/3 pyramidal neurons in an RNA interference model of methyl-CpG-binding protein 2 deficiency. J Neurosci (2009) 1.55

Correction of respiratory disorders in a mouse model of Rett syndrome. Proc Natl Acad Sci U S A (2010) 1.54

Epigenetic principles and mechanisms underlying nervous system functions in health and disease. Prog Neurobiol (2008) 1.53

Rett syndrome mutation MeCP2 T158A disrupts DNA binding, protein stability and ERP responses. Nat Neurosci (2011) 1.53

Reversal of phenotypes in MECP2 duplication mice using genetic rescue or antisense oligonucleotides. Nature (2015) 1.51

Functional recovery with recombinant human IGF1 treatment in a mouse model of Rett Syndrome. Proc Natl Acad Sci U S A (2014) 1.51

MeCP2-mediated transcription repression in the basolateral amygdala may underlie heightened anxiety in a mouse model of Rett syndrome. J Neurosci (2009) 1.50

An AT-hook domain in MeCP2 determines the clinical course of Rett syndrome and related disorders. Cell (2013) 1.49

Intact long-term potentiation but reduced connectivity between neocortical layer 5 pyramidal neurons in a mouse model of Rett syndrome. J Neurosci (2009) 1.47

Breathing disorders in Rett syndrome: progressive neurochemical dysfunction in the respiratory network after birth. Respir Physiol Neurobiol (2009) 1.46

The genetic basis of non-syndromic intellectual disability: a review. J Neurodev Disord (2010) 1.45

Pathogenic SYNGAP1 mutations impair cognitive development by disrupting maturation of dendritic spine synapses. Cell (2012) 1.42

Microglia development and function. Annu Rev Immunol (2014) 1.41

Recent advances in MeCP2 structure and function. Biochem Cell Biol (2009) 1.41

Safety, pharmacokinetics, and preliminary assessment of efficacy of mecasermin (recombinant human IGF-1) for the treatment of Rett syndrome. Proc Natl Acad Sci U S A (2014) 1.40

Readthrough of nonsense mutations in Rett syndrome: evaluation of novel aminoglycosides and generation of a new mouse model. J Mol Med (Berl) (2010) 1.40

Methyl-CpG binding proteins: specialized transcriptional repressors or structural components of chromatin? Cell Mol Life Sci (2008) 1.39

Long non-coding RNA modifies chromatin: epigenetic silencing by long non-coding RNAs. Bioessays (2011) 1.39

The role of MeCP2 in brain development and neurodevelopmental disorders. Curr Psychiatry Rep (2010) 1.37

From mTOR to cognition: molecular and cellular mechanisms of cognitive impairments in tuberous sclerosis. J Intellect Disabil Res (2009) 1.37

Complexities of Rett syndrome and MeCP2. J Neurosci (2011) 1.35

NMDA receptor regulation prevents regression of visual cortical function in the absence of Mecp2. Neuron (2012) 1.35

Genome-wide analysis reveals methyl-CpG-binding protein 2-dependent regulation of microRNAs in a mouse model of Rett syndrome. Proc Natl Acad Sci U S A (2010) 1.33

Downregulation of NR3A-containing NMDARs is required for synapse maturation and memory consolidation. Neuron (2009) 1.31

Experimental models of Rett syndrome based on Mecp2 dysfunction. Exp Biol Med (Maywood) (2011) 1.27

Systemic delivery of MeCP2 rescues behavioral and cellular deficits in female mouse models of Rett syndrome. J Neurosci (2013) 1.26

MeCP2 is critical for maintaining mature neuronal networks and global brain anatomy during late stages of postnatal brain development and in the mature adult brain. J Neurosci (2012) 1.25

Progress toward treatments for synaptic defects in autism. Nat Med (2013) 1.24

Excitatory/Inhibitory Balance and Circuit Homeostasis in Autism Spectrum Disorders. Neuron (2015) 1.24

Female Mecp2(+/-) mice display robust behavioral deficits on two different genetic backgrounds providing a framework for pre-clinical studies. Hum Mol Genet (2012) 1.23

The impact of MeCP2 loss- or gain-of-function on synaptic plasticity. Neuropsychopharmacology (2012) 1.22

Adult restoration of Shank3 expression rescues selective autistic-like phenotypes. Nature (2016) 1.21

DNA methylation impacts on learning and memory in aging. Neurobiol Aging (2007) 1.20

Cell-type-specific repression by methyl-CpG-binding protein 2 is biased toward long genes. J Neurosci (2014) 1.20

MeCP2 is critical within HoxB1-derived tissues of mice for normal lifespan. J Neurosci (2011) 1.19

The relationship of Rett syndrome and MECP2 disorders to autism. Dialogues Clin Neurosci (2012) 1.18

Transcriptional regulation and stabilization of left-right neuronal identity in C. elegans. Genes Dev (2009) 1.17

MeCP2 is required for normal development of GABAergic circuits in the thalamus. J Neurophysiol (2010) 1.17

Improved survival and reduced phenotypic severity following AAV9/MECP2 gene transfer to neonatal and juvenile male Mecp2 knockout mice. Mol Ther (2012) 1.16

Neurogenetics: advancing the "next-generation" of brain research. Neuron (2010) 1.16

Rett syndrome and the impact of MeCP2 associated transcriptional mechanisms on neurotransmission. Biol Psychiatry (2008) 1.16

Networking in autism: leveraging genetic, biomarker and model system findings in the search for new treatments. Neuropsychopharmacology (2011) 1.15

Convulsing toward the pathophysiology of autism. Brain Dev (2008) 1.15

Intellectual disability and autism spectrum disorders: causal genes and molecular mechanisms. Neurosci Biobehav Rev (2014) 1.14

Morphological and functional reversal of phenotypes in a mouse model of Rett syndrome. Brain (2012) 1.14

Rett syndrome: genes, synapses, circuits, and therapeutics. Front Psychiatry (2012) 1.14

Stress and the epigenetic landscape: a link to the pathobiology of human diseases? Nat Rev Genet (2010) 1.13

DLX5 and DLX6 expression is biallelic and not modulated by MeCP2 deficiency. Am J Hum Genet (2007) 1.13

Synaptic microcircuit dysfunction in genetic models of neurodevelopmental disorders: focus on Mecp2 and Met. Curr Opin Neurobiol (2011) 1.12

Long-lived epigenetic interactions between perinatal PBDE exposure and Mecp2308 mutation. Hum Mol Genet (2012) 1.12

Rett syndrome-causing mutations in human MeCP2 result in diverse structural changes that impact folding and DNA interactions. J Biol Chem (2008) 1.12

Rett syndrome: exploring the autism link. Arch Neurol (2011) 1.11

Genetic syndromes caused by mutations in epigenetic genes. Hum Genet (2013) 1.11

Epigenetic regulation of gene expression: how the genome integrates intrinsic and environmental signals. Nat Genet (2003) 25.57

DNA methylation landscapes: provocative insights from epigenomics. Nat Rev Genet (2008) 15.45

CpG islands and the regulation of transcription. Genes Dev (2011) 9.50

A novel CpG island set identifies tissue-specific methylation at developmental gene loci. PLoS Biol (2008) 7.95

Oxidative damage to methyl-CpG sequences inhibits the binding of the methyl-CpG binding domain (MBD) of methyl-CpG binding protein 2 (MeCP2). Nucleic Acids Res (2004) 7.50

CpG islands influence chromatin structure via the CpG-binding protein Cfp1. Nature (2010) 4.86

Embryonic lethal phenotype reveals a function of TDG in maintaining epigenetic stability. Nature (2011) 3.28

BLUEPRINT to decode the epigenetic signature written in blood. Nat Biotechnol (2012) 3.24

The role of MeCP2 in the brain. Annu Rev Cell Dev Biol (2011) 2.84

Enhanced CpG mutability and tumorigenesis in MBD4-deficient mice. Science (2002) 2.73

Cell type-specific DNA methylation at intragenic CpG islands in the immune system. Genome Res (2011) 2.51

CpG methylation is targeted to transcription units in an invertebrate genome. Genome Res (2007) 2.47

A temporal threshold for formaldehyde crosslinking and fixation. PLoS One (2009) 2.03

Up-regulation of glucocorticoid-regulated genes in a mouse model of Rett syndrome. Hum Mol Genet (2005) 1.87

Interaction between chromatin proteins MECP2 and ATRX is disrupted by mutations that cause inherited mental retardation. Proc Natl Acad Sci U S A (2007) 1.84

The major form of MeCP2 has a novel N-terminus generated by alternative splicing. Nucleic Acids Res (2004) 1.79

Rett syndrome mutations abolish the interaction of MeCP2 with the NCoR/SMRT co-repressor. Nat Neurosci (2013) 1.74

Kaiso-deficient mice show resistance to intestinal cancer. Mol Cell Biol (2006) 1.67

53BP1 exchanges slowly at the sites of DNA damage and appears to require RNA for its association with chromatin. J Cell Sci (2005) 1.62

DNA methylation and Rett syndrome. Hum Mol Genet (2003) 1.62

Base excision by thymine DNA glycosylase mediates DNA-directed cytotoxicity of 5-fluorouracil. PLoS Biol (2009) 1.61

Cfp1 integrates both CpG content and gene activity for accurate H3K4me3 deposition in embryonic stem cells. Genes Dev (2012) 1.55

Gene expression analysis exposes mitochondrial abnormalities in a mouse model of Rett syndrome. Mol Cell Biol (2006) 1.52

Deficiency of Mbd2 suppresses intestinal tumorigenesis. Nat Genet (2003) 1.44

Fas-associated death domain protein interacts with methyl-CpG binding domain protein 4: a potential link between genome surveillance and apoptosis. Proc Natl Acad Sci U S A (2003) 1.29

Systemic delivery of MeCP2 rescues behavioral and cellular deficits in female mouse models of Rett syndrome. J Neurosci (2013) 1.26

Mbd2 contributes to DNA methylation-directed repression of the Xist gene. Mol Cell Biol (2007) 1.22

MBD4 deficiency reduces the apoptotic response to DNA-damaging agents in the murine small intestine. Oncogene (2003) 1.21

Encoding and retrieval in a model of the hippocampal CA1 microcircuit. Hippocampus (2010) 1.19

Targeting of de novo DNA methylation throughout the Oct-4 gene regulatory region in differentiating embryonic stem cells. PLoS One (2010) 1.17

Enhanced hippocampal long-term potentiation and spatial learning in aged 11beta-hydroxysteroid dehydrogenase type 1 knock-out mice. J Neurosci (2007) 1.14

Morphological and functional reversal of phenotypes in a mouse model of Rett syndrome. Brain (2012) 1.14

The effect of interspecific oocytes on demethylation of sperm DNA. Proc Natl Acad Sci U S A (2004) 1.14

Genomic approaches reveal unexpected genetic divergence within Ciona intestinalis. J Mol Evol (2005) 1.14

OsPHF1 regulates the plasma membrane localization of low- and high-affinity inorganic phosphate transporters and determines inorganic phosphate uptake and translocation in rice. Plant Physiol (2011) 1.11

Postnatal inactivation reveals enhanced requirement for MeCP2 at distinct age windows. Hum Mol Genet (2012) 1.10

Disease modeling using embryonic stem cells: MeCP2 regulates nuclear size and RNA synthesis in neurons. Stem Cells (2012) 1.09

DNA repair gene Ercc1 is essential for normal spermatogenesis and oogenesis and for functional integrity of germ cell DNA in the mouse. Development (2003) 1.09

Molecular biology. MeCP2 repression goes nonglobal. Science (2003) 1.07

Reversibility of functional deficits in experimental models of Rett syndrome. Biochem Soc Trans (2010) 1.03

MMDiff: quantitative testing for shape changes in ChIP-Seq data sets. BMC Genomics (2013) 1.02

Testing for association between MeCP2 and the brahma-associated SWI/SNF chromatin-remodeling complex. Nat Genet (2006) 1.00

MeCP2 and other methyl-CpG binding proteins. Ment Retard Dev Disabil Res Rev (2002) 0.94

Il2 transcription unleashed by active DNA demethylation. Nat Immunol (2003) 0.93

Male infertility and DNA damage in Doppel knockout and prion protein/Doppel double-knockout mice. Am J Pathol (2004) 0.92

Putting the DNA back into DNA methylation. Nat Genet (2011) 0.92

Deletion of genes implicated in protecting the integrity of male germ cells has differential effects on the incidence of DNA breaks and germ cell loss. PLoS One (2007) 0.91

MBD4 deficiency does not increase mutation or accelerate tumorigenesis in mice lacking MMR. Oncogene (2004) 0.89

MBD2 is required for correct spatial gene expression in the gut. Mol Cell Biol (2007) 0.89

Cfp1 is required for gene expression-dependent H3K4 trimethylation and H3K9 acetylation in embryonic stem cells. Genome Biol (2014) 0.87

Characterization of proteinase-activated receptor 2 signalling and expression in rat hippocampal neurons and astrocytes. Neuropharmacology (2006) 0.87

MBD2-mediated transcriptional repression of the p14ARF tumor suppressor gene in human colon cancer cells. Pathobiology (2008) 0.86

OsDGL1, a homolog of an oligosaccharyltransferase complex subunit, is involved in N-glycosylation and root development in rice. Plant Cell Physiol (2012) 0.83

MBD2 deficiency does not accelerate p53 mediated lymphomagenesis. Oncogene (2005) 0.82

Identical sets of methylated and nonmethylated genes in Ciona intestinalis sperm and muscle cells. Epigenetics Chromatin (2013) 0.81

A unique DNA methylation signature defines a population of IFN-γ/IL-4 double-positive T cells during helminth infection. Eur J Immunol (2014) 0.80

Label-free imaging of thick tissue at 1550 nm using a femtosecond optical parametric generator. Opt Lett (2015) 0.77

Long-term home cage activity scans reveal lowered exploratory behaviour in symptomatic female Rett mice. Behav Brain Res (2013) 0.77

A computational study on plasticity during theta cycles at Schaffer collateral synapses on CA1 pyramidal cells in the hippocampus. Hippocampus (2014) 0.77

Spine head calcium as a measure of summed postsynaptic activity for driving synaptic plasticity. Neural Comput (2014) 0.75