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Human Molecular Genetics 2005 14(Review Issue 1):R19-R26; doi:10.1093/hmg/ddi102
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© The Author 2005. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org

MeCP2 in neurons: closing in on the causes of Rett syndrome

Isabel Martín Caballero and Brian Hendrich*

Institute for Stem Cell Research, Centre Development in Stem Cell Biology, School of Biological Sciences, University of Edinburgh, Edinburgh EH9 3JQ, UK

* To whom correspondence should be addressed. Tel: +44 131 650 5867; Fax: +44 131 650 7773; Email: brian.hendrich{at}ed.ac.uk

Received January 3, 2005; Revised January 25, 2005; Accepted February 23, 2005

The discovery in 1999 that Rett syndrome (RTT) is caused by mutations in a gene encoding the methyl-CpG-binding repressor protein MECP2 provided a significant breakthrough in the understanding of this devastating disease. The subsequent production of Mecp2 knockout mice 2 years later provided an experimental resource to better understand how mutations in the MECP2 gene result in RTT. This paper reviews the recent progress in understanding when and where MeCP2 function becomes important in the developing brain, why MeCP2 protein levels are crucial, which genes are normally silenced by MeCP2, and how misexpression of these targets might lead to the clinical manifestations of RTT.


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