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Article

IFNγ protects motor neurons from oxidative stress via enhanced global protein synthesis in FUS-associated amyotrophic lateral sclerosis.

Brain pathology (Zurich, Switzerland) - 1 Jan 2024

Assoni Amanda Faria, Guerrero Erika N, Wardenaar René, Oliveira Danyllo, Bakker Petra L, Alves Luciana M, Carvalho Valdemir M, Okamoto Oswaldo Keith, Zatz Mayana, Foijer Floris

Abstract excerpt

Amyotrophic lateral sclerosis type 6 (ALS6) is a familial subtype of ALS linked to Fused in Sarcoma (FUS) gene mutation. FUS mutations lead to decreased global protein synthesis, but the mechanism that drives this has not been established. Here, we used ALS6 patient-derived induced pluripotent stem cells (hIPSCs) to study the effect of the ALS6 FUSR521H mutation on the translation machinery in motor neurons...

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Source

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Identifiers

PMID: 37582053
DOI: 10.1111/bpa.13206
PMCID: PMC10711262

Provenance

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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