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[Heterogeneity in the regulation of cellular stress responses by FUS gene mutations associated with amyotrophic lateral sclerosis].

Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences - 28 Oct 2025

Yu Chenchen, Zeng Weiqian, Meekrathok Piyanat, Bu Yue, Wang Junling, Qiu Jian

Abstract excerpt

OBJECTIVES: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by the selective death of motor neurons, exhibiting marked clinical heterogeneity and lacking effective treatment. The etiology and pathogenic mechanisms remain incompletely understood. The FUS (fused in sarcoma) gene is one of the key causative genes in ALS. Pathogenic mutations in the encoded protein are...

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Amyotrophic Lateral Sclerosis
ATF4 gene
FUS gene
Mutation
Nuclear Localization Signals

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

RNA-Binding Protein FUS

Stress, Physiological

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Identifiers

PMID: 41656808
DOI: 10.11817/j.issn.1672-7347.2025.250211
PMCID: PMC12949858

Genes

FUS
ATF4
VDAC1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 31 Aug 2026.

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[Heterogeneity in the regulation of cellular stress responses by FUS gene mutations associated with amyotrophic lateral sclerosis].PMID 41656808Read ↗

Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granulesSemantic proximity Read ↗

Autosomal dominant inheritance of rapidly progressive amyotrophic lateral sclerosis due to a truncation mutation in the fused in sarcoma (FUS) gene.Semantic proximity Read ↗

Amyotrophic Lateral Sclerosis, FUS and Protein Synthesis Defects.Semantic proximity Read ↗

Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase.Semantic proximity Read ↗

Establishment of In Vitro FUS-Associated Familial Amyotrophic Lateral Sclerosis Model Using Human Induced Pluripotent Stem CellsSemantic proximity Read ↗

Yeast as a Model to Unravel Mechanisms Behind FUS Toxicity in Amyotrophic Lateral SclerosisSemantic proximity Read ↗

Functions of FUS/TLS From DNA Repair to Stress Response: Implications for ALSSemantic proximity Read ↗

ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUSSemantic proximity Read ↗

ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules.Semantic proximity Read ↗

Autophagy regulates amyotrophic lateral sclerosis-linked fused in sarcoma-positive stress granules in neurons.Semantic proximity Read ↗

FUS ALS neurons activate major stress pathways and reduce translation as an early protective mechanism against neurodegeneration.Semantic proximity Read ↗

ALS-Related Mutant FUS Protein Is Mislocalized to Cytoplasm and Is Recruited into Stress Granules of Fibroblasts from Asymptomatic FUS P525L Mutation Carriers.Semantic proximity Read ↗

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