Article
Global transcriptional and translational repression in human-embryonic-stem-cell-derived Rett syndrome neurons.
Cell stem cell - 3 Oct 2013
Li Yun, Wang Haoyi, Muffat Julien, Cheng Albert W, Orlando David A, Lovén Jakob, Kwok Show-Ming, Feldman Danielle A, Bateup Helen S, Gao Qing, Hockemeyer Dirk, Mitalipova Maisam, Lewis Caroline A, Vander Heiden Matthew G, Sur Mriganka, Young Richard A, Jaenisch Rudolf
Abstract excerpt
Rett syndrome (RTT) is caused by mutations of MECP2, a methyl CpG binding protein thought to act as a global transcriptional repressor. Here we show, using an isogenic human embryonic stem cell model of RTT, that MECP2 mutant neurons display key molecular and cellular features of this disorder. Unbiased global gene expression analyses demonstrate that MECP2 functions as a global activator in neurons but not in...
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