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

Publiceringsår: 2012
Språk: Engelska
Sidor: 1673-1680
Publikation/Tidskrift/Serie: Human molecular genetics
Volym: 21
Nummer: 8
Dokumenttyp: Artikel


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.



  • Medicine and Health Sciences


  • ISSN: 1460-2083

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