Article
GFAP Mutations in Astrocytes Impair Oligodendrocyte Progenitor Proliferation and Myelination in an hiPSC Model of Alexander Disease.
Cell stem cell - 2 Aug 2018
Li Li, Tian E, Chen Xianwei, Chao Jianfei, Klein Jeremy, Qu Qiuhao, Sun Guihua, Sun Guoqiang, Huang Yanzhou, Warden Charles D, Ye Peng, Feng Lizhao, Li Xinqiang, Cui Qi, Sultan Abdullah, Douvaras Panagiotis, Fossati Valentina, Sanjana Neville E, Riggs Arthur D, Shi Yanhong
Abstract excerpt
Alexander disease (AxD) is a leukodystrophy that primarily affects astrocytes and is caused by mutations in the astrocytic filament gene GFAP. While astrocytes are thought to have important roles in controlling myelination, AxD animal models do not recapitulate critical myelination phenotypes and it is therefore not clear how AxD astrocytes contribute to leukodystrophy. Here, we show that AxD patient iPSC-derived...
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