Journal article

Neurochemical changes in a mouse model of Rett syndrome: Changes over time and in response to perinatal choline nutritional supplementation

BC Ward, NH Kolodny, N Nag, JE Berger-Sweeney

Journal of Neurochemistry | WILEY | Published : 2009

Abstract

Rett syndrome (RTT), the second leading cause of mental retardation in girls, is caused by mutations in the X-linked gene for methyl-CpG-binding protein 2 (MeCP2), a transcriptional repressor. In addition to well-documented neuroanatomical and behavioral deficits, RTT is characterized by reduced markers of cholinergic activity and general neuronal health. Previously, we have shown that early postnatal choline (Cho) supplementation improves behavioral and neuroanatomical symptoms in a mouse model of RTT (Mecp21lox mice). In this study, we use NMR spectroscopy to quantify the relative amounts of Cho, Glutamate (Glu), Glutamine (Gln), and N-acetyl aspartate (NAA) in the brains of wild type and ..

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University of Melbourne Researchers

Grants

Funding Acknowledgements

We would like to express our thanks to Dr Urs Berger for genotyping to Ms Brittany Yerby for development of the metabolite extraction procedure, Dr Laura Schaevitz for suggestions to the manuscript, and to Ms Patience Carey, Ms Ginny Quinan, and Ms Valerie LePage for their excellent care of our animals. Financial support for this project was provided from the following sources: National Science Foundation, International Rett Syndrome Association (IRSA), Wellesley College Faculty Award and Staley Fund, and the Howard Hughes Medical Institute Grant to Wellesley College.