Pharmacological interference with the glucocorticoid system influences symptoms and lifespan in a mouse model of Rett syndrome.
(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.
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/2273756
- author
- Braun, Sebastian LU ; Kottwitz, Denise and Nuber, Ulrike LU
- organization
- publishing date
- 2012
- 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
- pmid:22186023
- 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
- 2016-04-04 08:05:28
- date last changed
- 2022-07-24 18:59:56
@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}}, doi = {{10.1093/hmg/ddr602}}, volume = {{21}}, year = {{2012}}, }