Article
Dysregulation of BRD4 Function Underlies the Functional Abnormalities of MeCP2 Mutant Neurons.
Molecular cell - 2 Jul 2020
Xiang Yangfei, Tanaka Yoshiaki, Patterson Benjamin, Hwang Sung-Min, Hysolli Eriona, Cakir Bilal, Kim Kun-Yong, Wang Wanshan, Kang Young-Jin, Clement Ethan M, Zhong Mei, Lee Sang-Hun, Cho Yee Sook, Patra Prabir, Sullivan Gareth J, Weissman Sherman M, Park In-Hyun
Abstract excerpt
Rett syndrome (RTT), mainly caused by mutations in methyl-CpG binding protein 2 (MeCP2), is one of the most prevalent intellectual disorders without effective therapies. Here, we used 2D and 3D human brain cultures to investigate MeCP2 function. We found that MeCP2 mutations cause severe abnormalities in human interneurons (INs). Surprisingly, treatment with a BET inhibitor, JQ1, rescued the molecular and...
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