pubmed-article:18223199 | rdf:type | pubmed:Citation | lld:pubmed |
pubmed-article:18223199 | lifeskim:mentions | umls-concept:C0282641 | lld:lifeskim |
pubmed-article:18223199 | lifeskim:mentions | umls-concept:C0015780 | lld:lifeskim |
pubmed-article:18223199 | lifeskim:mentions | umls-concept:C0026809 | lld:lifeskim |
pubmed-article:18223199 | lifeskim:mentions | umls-concept:C0027882 | lld:lifeskim |
pubmed-article:18223199 | lifeskim:mentions | umls-concept:C1417098 | lld:lifeskim |
pubmed-article:18223199 | lifeskim:mentions | umls-concept:C0085140 | lld:lifeskim |
pubmed-article:18223199 | lifeskim:mentions | umls-concept:C0004927 | lld:lifeskim |
pubmed-article:18223199 | lifeskim:mentions | umls-concept:C0332161 | lld:lifeskim |
pubmed-article:18223199 | lifeskim:mentions | umls-concept:C0011209 | lld:lifeskim |
pubmed-article:18223199 | lifeskim:mentions | umls-concept:C0599894 | lld:lifeskim |
pubmed-article:18223199 | lifeskim:mentions | umls-concept:C1521840 | lld:lifeskim |
pubmed-article:18223199 | pubmed:issue | 10 | lld:pubmed |
pubmed-article:18223199 | pubmed:dateCreated | 2008-5-1 | lld:pubmed |
pubmed-article:18223199 | pubmed:abstractText | Rett syndrome is an X-linked neurological condition affecting almost exclusively girls that is caused by mutations of the MECP2 gene. Recent studies have shown that transgenic delivery of MeCP2 function to Mecp2-deficient male mice can improve their Rett-like behavior. However, as the brain of a Rett girl contains a mosaic of MeCP2 expressing and non-expressing neurons, and the over-expression of MeCP2 in neurons can induce a severe progressive neurological phenotype, testing whether functional rescue can be achieved by gene re-introduction strategies in a female model of Rett syndrome is warranted. To address this, we generated transgenic mice expressing an epitope-tagged Mecp2 transgene in forebrain neurons. These mice over-express MeCP2 protein at about 1.6 times normal levels in cortex and develop impaired motor behavior by 9-12 months of age. To test whether forebrain-targeted MeCP2 restoration would improve behavior in female Mecp2(-/+) mice, we crossed these transgenics with Mecp2(-/+) mice and examined the behavioral properties of the female rescue mice for 1 year. These assessments revealed that the diminished rearing activity, impaired mobility and the diminished locomotive activity of female Mecp2(-/+) mice were restored to wild-type levels in the rescue mice. These results show that improvement of Rett-like behavior can be achieved in Mecp2(-/+) females by targeted gene re-introduction without inducing deficits relating to MeCP2 over-expression. | lld:pubmed |
pubmed-article:18223199 | pubmed:language | eng | lld:pubmed |
pubmed-article:18223199 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:18223199 | pubmed:citationSubset | IM | lld:pubmed |
pubmed-article:18223199 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:18223199 | pubmed:status | MEDLINE | lld:pubmed |
pubmed-article:18223199 | pubmed:month | May | lld:pubmed |
pubmed-article:18223199 | pubmed:issn | 1460-2083 | lld:pubmed |
pubmed-article:18223199 | pubmed:author | pubmed-author:BrotchieJonat... | lld:pubmed |
pubmed-article:18223199 | pubmed:author | pubmed-author:JugloffDenis... | lld:pubmed |
pubmed-article:18223199 | pubmed:author | pubmed-author:EubanksJames... | lld:pubmed |
pubmed-article:18223199 | pubmed:author | pubmed-author:LoganRichardR | lld:pubmed |
pubmed-article:18223199 | pubmed:author | pubmed-author:VisanjiNaomi... | lld:pubmed |
pubmed-article:18223199 | pubmed:author | pubmed-author:VandammeKatri... | lld:pubmed |
pubmed-article:18223199 | pubmed:issnType | Electronic | lld:pubmed |
pubmed-article:18223199 | pubmed:day | 15 | lld:pubmed |
pubmed-article:18223199 | pubmed:volume | 17 | lld:pubmed |
pubmed-article:18223199 | pubmed:owner | NLM | lld:pubmed |
pubmed-article:18223199 | pubmed:authorsComplete | Y | lld:pubmed |
pubmed-article:18223199 | pubmed:pagination | 1386-96 | lld:pubmed |
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pubmed-article:18223199 | pubmed:year | 2008 | lld:pubmed |
pubmed-article:18223199 | pubmed:articleTitle | Targeted delivery of an Mecp2 transgene to forebrain neurons improves the behavior of female Mecp2-deficient mice. | lld:pubmed |
pubmed-article:18223199 | pubmed:affiliation | Division of Genetics and Development, Toronto Western Research Institute, Toronto, Ontario, Canada. | lld:pubmed |
pubmed-article:18223199 | pubmed:publicationType | Journal Article | lld:pubmed |
pubmed-article:18223199 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
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