MECP2 impairs neuronal structure by regulating KIBRA
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- 作者：Alison A. Williamsa ; d ; awilli27@uw.edu" class="auth_mail" title="E-mail the corresponding author ; Robin Whiteb ; Ashley Siniardc ; Jason Corneveauxc ; Matt Huentelmanc ; Carsten Duchd
- 关键词：MECP2 ; Methyl-CpG-binding protein 2 ; RTT ; Rett syndrome ; MDS ; MECP2 duplication syndrome ; hMECP2 ; human MECP2 ; 5&prime ; Mc ; 5-mehtylcytosine ; MBD ; methyl binding domain ; hR106W ; human MECP2 variant R106W ; qRT-PCR ; quantitative real-time polymerase chain reaction ; Mecp2&minus ; /Y ; Mecp2 null mouse model ; mMecp2 ; mouse Mecp2 ; mKibra ; mouse Kibra ; dkibra ; Drosophila kibra
- 刊名：Neurobiology of Disease
- 出版年：2016
- 出版时间：July 2016
- 年：2016
- 卷：91
- 期：Complete
- 页码：284-291
- 全文大小：601 K
文摘
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Expression of hMECP2 in Drosophila CNS impairs dendrites and increases kibra.
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Mecp2 gain-of-function effects are validated in mouse primary neuron culture.
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Mecp2−/Y mice show decreased Kibra levels in cortex, cerebellum, and hippocampus.
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Kibra knockdown rescues dendritic defects caused by hMECP2 expression in Drosophila.