Mecp2 deficiency leads to delayed maturation and altered gene expression in hippocampal neurons

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Mecp2 deficiency leads to delayed maturation and altered gene expression in hippocampal neurons.

It is well known that Rett Syndrome, a severe postnatal childhood neurological disorder, is mostly caused by mutations in the MECP2 gene. However, how deficiencies in MeCP2 contribute to the neurological dysfunction of Rett Syndrome is not clear. We aimed to resolve the role of MeCP2 epigenetic regulation in postnatal brain development in an Mecp2-deficient mouse model. We found that, while Mec...

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Mecp2 deficiency is associated with learning and cognitive deficits and altered gene activity in the hippocampal region of mice.

Rett syndrome (RTT) is a debilitating neurological condition associated with mutations in the X-linked MECP2 gene, where apparently normal development is seen prior to the onset of cognitive and motor deterioration at 6-18 months of life. A targeted deletion of the methyl-CpG-binding domain (MBD) coding region and disruption of mRNA splicing was introduced in the mouse, resulting in a complete ...

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MeCP2 Deficiency Leads to Loss of Glial Kir4.1

Rett syndrome (RTT) is an X-linked neurodevelopmental disorder usually caused by mutations in methyl-CpG-binding protein 2 (MeCP2). RTT is typified by apparently normal development until 6-18 mo of age, when motor and communicative skills regress and hand stereotypies, autonomic symptoms, and seizures present. Restoration of MeCP2 function selectively to astrocytes reversed several deficits in ...

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Epigenetic regulation of neuronal maturation : the effect of MeCP2 and MicroRNAs on the maturation of hippocampal neurons

OF DISSERTATION Submitted in Partial Fulfillment of the Requirements for the Degree of The University of New Mexico Albuquerque, New Mexico

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ژورنال

عنوان ژورنال: Neurobiology of Disease

سال: 2007

ISSN: 0969-9961

DOI: 10.1016/j.nbd.2007.04.005