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

First Author  Braun S Year  2012
Journal  Hum Mol Genet Volume  21
Issue  8 Pages  1673-80
PubMed ID  22186023 Mgi Jnum  J:181897
Mgi Id  MGI:5314325 Doi  10.1093/hmg/ddr602
Citation  Braun S, et al. (2012) Pharmacological interference with the glucocorticoid system influences symptoms and lifespan in a mouse model of Rett syndrome. Hum Mol Genet 21(8):1673-80
abstractText  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 an 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 an 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.
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