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Neurobiol Dis ; 38(1): 8-16, 2010 Apr.
Article in English | MEDLINE | ID: mdl-20045053

ABSTRACT

Rett syndrome is a pediatric neurological condition caused by mutations of the gene encoding the transcriptional regulator MECP2. In this study, we examined cortical and hippocampal electroencephalographic (EEG) activity in male and female MeCP2-deficient mice at symptomatic stages during different behavioral states. During acute sleep, MeCP2-deficient mice displayed normal delta-like activity in cortex and sharp-wave activity in hippocampus. However, when the mice were awake but immobile, abnormal spontaneous, rhythmic EEG discharges of 6-9 Hz were readily detected in the somatosensory cortex. During exploratory activity, MeCP2-deficient mice displayed clear theta rhythm activity in hippocampus, but its peak frequency was significantly attenuated compared to wild type. Collectively, these findings indicate that a deficiency in MeCP2 function in mice leads to alterations in EEG activity with similarities to what has been observed clinically in Rett syndrome patients.


Subject(s)
Cerebral Cortex/physiopathology , Evoked Potentials/genetics , Hippocampus/physiopathology , Methyl-CpG-Binding Protein 2/genetics , Rett Syndrome/physiopathology , Action Potentials/genetics , Animals , Cerebral Cortex/metabolism , Disease Models, Animal , Electroencephalography , Exploratory Behavior/physiology , Female , Genetic Predisposition to Disease/genetics , Hippocampus/metabolism , Male , Mice , Mice, Knockout , Rett Syndrome/genetics , Rett Syndrome/metabolism , Theta Rhythm
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