Developmental abnormalities of cortical interneurons precede symptoms onset in a mouse model of Rett syndrome

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Developmental abnormalities of cortical interneurons precede symptoms onset in a mouse model of Rett syndrome.

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Rett syndrome (RTT) is a severe neurological disorder caused by mutations in the X-linked MECP2 gene, which encodes a methyl-CpG binding transcriptional repressor. Using the Mecp2-null mouse (an animal model for RTT) and differential display, we found that mice with neurological symptoms overexpress the nuclear gene for ubiquinol-cytochrome c reductase core protein 1 (Uqcrc1). Chromatin immunop...

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Rett syndrome (RTT) is a neuro-developmental disorder caused by loss of function of Mecp2--methyl-CpG-binding protein 2--an epigenetic factor controlling DNA transcription. In mice, removal of Mecp2 in the forebrain recapitulates most of behavioral deficits found in global Mecp2 deficient mice, including amygdala-related hyper-anxiety and lack of social interaction, pointing a role of Mecp2 in ...

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ژورنال

عنوان ژورنال: Journal of Neurochemistry

سال: 2014

ISSN: 0022-3042

DOI: 10.1111/jnc.12803