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Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS.
Claire E Hall, Zhi Yao, Minee Choi, Giulia E Tyzack, Andrea Serio, Raphaelle Luisier, Jasmine Harley, Elisavet Preza, Charlie Arber, Sarah J Crisp, P Marc D Watson, Dimitri M Kullmann, Andrey Y Abramov, Selina Wray, Russell Burley, Samantha HY Loh, L Miguel Martins, Molly M Stevens, Nicholas M Luscombe, Christopher R Sibley, Andras Lakatos, Jernej Ule, Sonia Gandhi, Rickie Patani
Nov 29, 2018
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...
amyotrophic lateral sclerosis (ALS) astrocytes (ACs) Disease Modeling induced pluripotent stem cells (iPSCs) motor neurons (MNs) Amyotrophic Lateral Sclerosis Astrocytes Cell Survival DNA-Binding Proteins Endoplasmic Reticulum Endoplasmic reticulum stress Humans Induced Pluripotent Stem Cells Membrane potential Mitochondrial Mitochondria Models Biological Motor Neurons Mutation Nerve Degeneration Neurogenesis Oxidative Stress Phenotype Synapses Valosin Containing Protein