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

Braun, Sebastian LU ; Kottwitz, Denise and Nuber, Ulrike LU (2012) In Human Molecular Genetics 21(8). p.1673-1680
Abstract
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.
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Human Molecular Genetics
volume
21
issue
8
pages
1673 - 1680
publisher
Oxford University Press
external identifiers
  • wos:000302302400001
  • pmid:22186023
  • scopus:84859233352
ISSN
0964-6906
DOI
10.1093/hmg/ddr602
language
English
LU publication?
yes
id
41a60c89-75bc-4256-8ef8-056825eb6cb2 (old id 2273756)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/22186023?dopt=Abstract
date added to LUP
2012-01-03 20:47:43
date last changed
2017-01-01 07:34:56
@article{41a60c89-75bc-4256-8ef8-056825eb6cb2,
  abstract     = {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.},
  author       = {Braun, Sebastian and Kottwitz, Denise and Nuber, Ulrike},
  issn         = {0964-6906},
  language     = {eng},
  number       = {8},
  pages        = {1673--1680},
  publisher    = {Oxford University Press},
  series       = {Human Molecular Genetics},
  title        = {Pharmacological interference with the glucocorticoid system influences symptoms and lifespan in a mouse model of Rett syndrome.},
  url          = {http://dx.doi.org/10.1093/hmg/ddr602},
  volume       = {21},
  year         = {2012},
}