2014
DOI: 10.1016/j.mcn.2014.01.005
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MeCP2 is required for activity-dependent refinement of olfactory circuits

Abstract: Methyl CpG binding protein 2 (MeCP2) is a structural chromosomal protein involved in the regulation of gene expression. Alterations in the levels of MeCP2 have been related to neurodevelopmental disorders. Studies in mouse models of MeCP2 deficiency have demonstrated that this protein is important for neuronal maturation, neurite complexity, synaptogenesis, and synaptic plasticity. However, the mechanisms by which MeCP2 dysfunction leads to neurodevelopmental defects, and the role of activity, remain unclear, … Show more

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Cited by 30 publications
(30 citation statements)
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References 100 publications
(159 reference statements)
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“…Patients with Rett Syndrome are typically characterized by a normal development at birth and subsequent failure to thrive leading to microcephaly and intellectual disability that develops with age. As a result, Rett Syndrome is thought to be caused by experience-dependent loss of neuronal function, which would correlate with data suggesting that MECP2 regulates activity dependent gene expression 10,13,37 . The microcephaly has been proposed to be a function of decreased nuclear size and dendritic arborization of affected neurons 13,22 .…”
Section: Discussionsupporting
confidence: 53%
“…Patients with Rett Syndrome are typically characterized by a normal development at birth and subsequent failure to thrive leading to microcephaly and intellectual disability that develops with age. As a result, Rett Syndrome is thought to be caused by experience-dependent loss of neuronal function, which would correlate with data suggesting that MECP2 regulates activity dependent gene expression 10,13,37 . The microcephaly has been proposed to be a function of decreased nuclear size and dendritic arborization of affected neurons 13,22 .…”
Section: Discussionsupporting
confidence: 53%
“…It is also worth noting that MECP2 mutations cause structural changes of brain neurons, often described as more dense and packed (Jentarra et al, 2010). Recent findings have also documented pronounced changes in sensory olfactory and trigeminal neurons (Degano, Park, Penati, Li, & Ronnett, 2014) providing further evidence of the importance of MeCP2's role in activity-dependent maturation of sensory circuitry. Although speculative, it may be that the complex morphological ENF observations reflect dorsal root ganglion (DRG) neuron abnormalities, as suggested also by Bhattacherjee et al (2017) in their documented pattern of somatosensory deficits associated with MECP2 mutation, that appear to be cell-autonomous to primary sensory neurons of the DRG.…”
Section: Discussionmentioning
confidence: 89%
“…Their families report often that they are using eye contact as communication method and therefore eye tracking systems are a promising method to improve communication.Molecular/histological data: MECP2 deficiency induces also an imbalance in glutamatergic/GABAergic innervation in the olfactory bulb. The excitation in MECP2 KO mice is reduced and there is generally an imbalance between excitatory and inhibitory pathways observed leading to premature death of olfactory neurons in RTT mice models [121]. MECP2 seems to regulate the activity dependent transcriptional responses in olfactory sensory neurons the same way as in central nervous system and model systems [123].…”
Section: Current Gaps In Understanding Rtt Pathwaysmentioning
confidence: 99%