Article
Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS.
Cell reports - 30 May 2017
Hall Claire E, Yao Zhi, Choi Minee, Tyzack Giulia E, Serio Andrea, Luisier Raphaelle, Harley Jasmine, Preza Elisavet, Arber Charlie, Crisp Sarah J, Watson P Marc D, Kullmann Dimitri M, Abramov Andrey Y, Wray Selina, Burley Russell, Loh Samantha H Y, Martins L Miguel, Stevens Molly M, Luscombe Nicholas M, Sibley Christopher R, Lakatos Andras, Ule Jernej, Gandhi Sonia, Patani Rickie
Abstract excerpt
Motor neurons (MNs) and astrocytes (ACs) are implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS), but their interaction and the sequence of molecular events leading to MN death remain unresolved. Here, we optimized directed differentiation of induced pluripotent stem cells (iPSCs) into highly enriched (> 85%) functional populations of spinal cord MNs and ACs. We identify significantly increased...
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