Article
Isogenic FUS-eGFP iPSC Reporter Lines Enable Quantification of FUS Stress Granule Pathology that Is Rescued by Drugs Inducing Autophagy.
Stem cell reports - 13 Feb 2018
Marrone Lara, Poser Ina, Casci Ian, Japtok Julia, Reinhardt Peter, Janosch Antje, Andree Cordula, Lee Hyun O, Moebius Claudia, Koerner Ellen, Reinhardt Lydia, Cicardi Maria Elena, Hackmann Karl, Klink Barbara, Poletti Angelo, Alberti Simon, Bickle Marc, Hermann Andreas, Pandey Udai Bhan, Hyman Anthony A, Sterneckert Jared L
Abstract excerpt
Perturbations in stress granule (SG) dynamics may be at the core of amyotrophic lateral sclerosis (ALS). Since SGs are membraneless compartments, modeling their dynamics in human motor neurons has been challenging, thus hindering the identification of effective therapeutics. Here, we report the generation of isogenic induced pluripotent stem cells carrying wild-type and P525L FUS-eGFP. We demonstrate that...
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