MeCP2 in Rett syndrome: transcriptional repressor or chromatin architectural protein?

被引:34
作者
Chadwick, Lisa Helbling [1 ]
Wade, Paul A. [1 ]
机构
[1] Natl Inst Environm Hlth Sci, Lab Mol Carcinogenesis, Res Triangle Pk, NC 27709 USA
关键词
D O I
10.1016/j.gde.2007.02.003
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
Rett syndrome is a progressive neurological disorder caused by mutations in the methyl-DNA binding protein MeCP2. The longstanding model depicting MeCP2 as a transcriptional repressor predicts that the Rett syndrome phenotype probably results from misregulation of MeCP2 target genes. Somewhat unexpectedly, the identification of such targets has proven challenging. The recent identification of two MeCP2 targets, BDNF and DLX5, are suggestive of two very different roles for this protein - one as a classical repressor protein, and the other as a mediator of a complex, specialized chromatin structure.
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收藏
页码:121 / 125
页数:5
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