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Dysregulated cortical synaptic plasticity under methyl-CpG binding protein 2 deficiency and its implication in motor
Wei-Jia Zhang1, Ling-Ling Shi1, Li Zhang2
1GHM Institute of CNS Regeneration, Jinan University, Guangzhou 510632, Guangdong Province, China.
Abstract:
Caused by the mutation of methyl-CpG binding protein 2 (MeCP2), Rett syndrome leads to a battery of severe neural dysfunctions including the regression of motor coordination and motor learning. Current understanding has revealed the motor cortex as the critical region mediating voluntary movement. In this review article, we will summarize major findings from human patients and animal models regarding the cortical synaptic plasticity under the regulation of MeCP2. We will also discuss how mutation of MeCP2 leads to the disruption of cortical circuitry homeostasis to cause motor deficits. Lastly, potential values of physical exercise and neuromodulation approaches to recover neural plasticity and motor function will be evaluated. All of this evidence may help to accelerate timely diagnosis and effective interventions for Rett syndrome patients.
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