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Pharmacological interference with the glucocorticoid system influences symptoms and lifespan in a mouse model of Rett syndrome.

Author

Summary, in English

Rett syndrome (RTT) is caused by loss-of-function mutations in the X-linked gene MECP2 coding for methyl CpG-binding protein 2 (MeCP2). This protein can act as transcriptional repressor and we showed in a previous study that glucocorticoid-inducible genes are up-regulated in a RTT mouse model and that these genes are direct MeCP2 targets. Here, we report that pharmacological intervention with the glucocorticoid system has an impact on the symptoms and lifespan in a RTT mouse model. Our data support a functional implication of the stress hormone system in RTT and suggest this hormone system as potential therapeutic target.

Publishing year

2012

Language

English

Pages

1673-1680

Publication/Series

Human Molecular Genetics

Volume

21

Issue

8

Document type

Journal article

Publisher

Oxford University Press

Topic

  • Medical Genetics

Status

Published

ISBN/ISSN/Other

  • ISSN: 0964-6906