by Holly N. Cukier, Alma M. Perez, Ann L. Collins, Zhaolan Zhou, Huda Y. Zoghbi, Juan Botas
Author Summary
Rett syndrome (RTT) is a progressive neurodevelopmental disorder that affects girls early in childhood and is caused by mutations in the MECP2 gene. Loss of MeCP2 function can also lead to clinically distinct conditions characterized by autism, learning disability, and mental retardation. Remarkably, increased levels of MeCP2 leads to related neurological disorders and mental retardation as well. These data emphasize the critical importance of regulating MeCP2 protein levels for normal post-natal development and function of the nervous system. MeCP2 is a protein that associates with chromatin and is thought to modulate gene expression. We have generated Drosophila that overexpress human MeCP2 to investigate the possibility that adjusting the activity of other genes may compensate for altered levels of MeCP2. In support of this hypothesis, we found a variety of modifier genes, including chromatin remodeling genes, that are able to ameliorate and/or aggravate the consequences of MeCP2 overexpression. These findings open the possibility of therapeutic avenues for RTT and related neuropsychiatric disorders by targeting proteins that are possibly easier to manipulate than MeCP2 itself. (Source: PLoS Genetics)
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Original source: http://feeds.feedburner.com/~r/plosgenetics/NewArticles/~3/384122146/info%3Adoi%2F10.1371%2Fjournal.pgen.1000179
