293 results found
    1. Neuroscience

    Rett-causing mutations reveal two domains critical for MeCP2 function and for toxicity in MECP2 duplication syndrome mice

    Laura Dean Heckman et al.
    Transgenic mice with Rett-causing mutations in MeCP2 reveal that a basic cluster in the C-terminus of the protein binds DNA and that both the methyl-CpG binding domain and the transcriptional repression domain are necessary to elicit toxicity in MECP2 duplication syndrome.
    1. Neuroscience

    Impaired spatial memory codes in a mouse model of Rett syndrome

    Sara E Kee et al.
    Hypersynchrony in a mouse model of Rett syndrome impairs ripple-dependent memory consolidation and leads to a decrease in experience-dependent refinement of place cell activities.
    1. Developmental Biology

    Mechanism and consequence of abnormal calcium homeostasis in Rett syndrome astrocytes

    Qiping Dong et al.
    Increased spontaneous calcium activity in Rett syndrome astrocytes is a key cell-autonomous phenotype that affects synaptic function and network activity.
    1. Neuroscience

    Restoration of Mecp2 expression in GABAergic neurons is sufficient to rescue multiple disease features in a mouse model of Rett syndrome

    Kerstin Ure et al.
    Genetically restoring Mecp2 expression only in GABAergic neurons in a mouse model of Rett syndrome improves inhibitory signaling, extends lifespan and rescues most but not all behavioral deficits.
    1. Neuroscience

    Astrocytic modulation of excitatory synaptic signaling in a mouse model of Rett syndrome

    Benjamin Rakela et al.
    Astrocytes impact neuronal signaling in brain dependent on status of the transcriptional repressor, Methyl CpG Binding Protein 2.
    1. Chromosomes and Gene Expression
    2. Neuroscience

    Losing Dnmt3a dependent methylation in inhibitory neurons impairs neural function by a mechanism impacting Rett syndrome

    Laura A Lavery et al.
    Dnmt3a regulates gene expression in inhibitory neurons by writing all mCH and some mCG, and MeCP2 reads some of these mCH sites driving a portion of these gene expression changes.
    1. Neuroscience

    Whole brain delivery of an instability-prone Mecp2 transgene improves behavioral and molecular pathological defects in mouse models of Rett syndrome

    Mirko Luoni et al.
    Global brain transduction of the instability-prone Mecp2 transgene by systemic AAV-PHP.eB administration is safe and effective in protecting male and female Mecp2 mutant mice from the Rett syndrome disease phenotype.
    1. Neuroscience

    Lovastatin fails to improve motor performance and survival in methyl-CpG-binding protein2-null mice

    Claudia Villani et al.
    Lovastatin ability to rescue motor deficits and survival in a mouse model of Rett syndrome is strongly influenced by the genetic background.
    1. Neuroscience

    Forniceal deep brain stimulation induces gene expression and splicing changes that promote neurogenesis and plasticity

    Amy E Pohodich et al.
    Forniceal deep brain stimulation is a promising treatment for several neuropsychiatric disorders as it upregulates synaptic and neurogenesis-associated genes, normalizes genes misregulated in Rett syndrome mice, and regulates genes altered in intellectual disability and major depression.
    1. Neuroscience

    Loss of MeCP2 disrupts cell autonomous and autocrine BDNF signaling in mouse glutamatergic neurons

    Charanya Sampathkumar et al.
    In mouse models of Rett syndrome, the impaired cell autonomous BDNF feed forward signaling pathway results in abnormalities in neurite outgrowth and synapse formation in excitatory neurons.

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