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Neuron. 2001 Jun;30(3):649-52.

Methylated cytosine and the brain: a new base for neuroscience.

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Friedrich Miescher Institute for Biomedical Research, 4058, Basel, Switzerland.


Methylation of genomic CpG residues is crucial for proper neuronal function. Rett syndrome, a common form of mental retardation, is associated with mutations in the gene encoding MeCP2, a methyl CpG binding protein linked to transcriptional repression. Gene knockouts of mouse Mecp2 have reproduced key aspects of the disease. A CNS-restricted knockout of Dnmt1, encoding the enzyme that maintains CpG methylation patterns, results in loss of mutant neurons and glia.

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