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Human Molecular Genetics Advance Access published online on August 19, 2003
Human Molecular Genetics, doi:10.1093/hmg/ddg286
© 2003 by Oxford University Press
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©2003 Oxford University Press

Article

DNA Methylation and Rett syndrome

Skirmantas Kriaucionis 1 and Adrian Bird 1*

1 Wellcome Trust Centre for Cell Biology, University of Edinburgh, The King's Buildings, Edinburgh EH9 3JR, Scotland, U.K.

* To whom correspondence should be addressed. E-mail: A.Bird{at}ed.ac.uk.


  Abstract

Methylation of cytosine in human DNA has been studied for over 60 years, but has only recently been confirmed as an important player in human disease. Rett Syndrome is a neurological disorder caused by mutations in the MeCP2 protein, which has been shown to bind methylated DNA and repress transcription. This review will focus on experiments addressing the basic properties of MeCP2 and on mouse models of Rett Syndrome that are starting to yield insights into this condition.


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