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A methyl-CpG-binding protein 2-enhanced green fluorescent protein reporter mouse model provides a new tool for studying the neuronal basis of Rett syndrome.

Schmid, Ralf S and Tsujimoto, Naomi and Qu, Qiang and Lei, Hong and Li, En and Chen, Taiping and Blaustein, Cecile (2008) A methyl-CpG-binding protein 2-enhanced green fluorescent protein reporter mouse model provides a new tool for studying the neuronal basis of Rett syndrome. Neuroreport, 19 (4). pp. 393-398. ISSN 0959-4965

Abstract

Rett syndrome, a pervasive X-linked neurodevelopmental disorder in young girls, is caused by loss-of-function mutations in the gene that encodes the transcriptional repressor methyl-CpG-binding protein 2 (MeCP2). Mecp2-knockout mice phenocopy the major symptoms found in human patients and have advanced our understanding of the function of MeCP2 and mechanism of Rett syndrome. To study the behavior of the MeCP2 protein in vivo, we have generated a knock-in reporter mouse model that expresses MeCP2-enhanced green fluorescent protein (EGFP) fusion protein instead of endogenous MeCP2. Here we show that expression of the fusion protein in the brain remarkably mirrors endogenous MeCP2 expression in all temporal and spatial aspects. This mouse model may be a valuable tool for studying Rett syndrome and for developing therapies.

Item Type: Article
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Additional Information: author can archive post-print (ie final draft post-refereeing); Post-print may be deposited in personal website, university's institutional repository or employers intranet; Publisher's version/PDF cannot be used; Must include statement that it is not the final published version
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Date Deposited: 14 Dec 2009 14:04
Last Modified: 31 Jan 2013 01:25
URI: https://oak.novartis.com/id/eprint/187

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