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
Links
Document type
Journal article
Publisher
Oxford University Press
Topic
- Medical Genetics
Status
Published
ISBN/ISSN/Other
- ISSN: 0964-6906