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Article

Homozygous ALS-linked FUS P525L mutations cell- autonomously perturb transcriptome profile and chemoreceptor signaling in human iPSC microglia.

Stem cell reports - 8 Mar 2022

Kerk Sze Yen, Bai Yu, Smith Janell, Lalgudi Pranav, Hunt Charleen, Kuno Junko, Nuara John, Yang Tao, Lanza Kathryn, Chan Newton, Coppola Angel, Tang Qian, Espert Jennifer, Jones Henderson, Fannell Casey, Zambrowicz Brian, Chiao Eric

Abstract excerpt

Amyotrophic lateral sclerosis is a fatal disease pathologically typified by motor and cortical neurodegeneration as well as microgliosis. The FUS P525L mutation is highly penetrant and causes ALS cases with earlier disease onset and more aggressive progression. To date, how P525L mutations may affect microglia during ALS pathogenesis had not been explored. In this study, we engineered isogenic control and P525L...

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Identifiers

PMID: 35120624
DOI: 10.1016/j.stemcr.2022.01.004
PMCID: PMC9039753

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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