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Article

ALS-linked FUS exerts a gain of toxic function involving aberrant p38 MAPK activation.

Scientific reports - 8 Mar 2017

Sama Reddy Ranjith K, Fallini Claudia, Gatto Rodolfo, McKeon Jeanne E, Song Yuyu, Rotunno Melissa S, Penaranda Saul, Abdurakhmanov Izrail, Landers John E, Morfini Gerardo, Brady Scott T, Bosco Daryl A

Abstract excerpt

Mutations in Fused in Sarcoma/Translocated in Liposarcoma (FUS) cause familial forms of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by progressive axonal degeneration mainly affecting motor neurons. Evidence from transgenic mouse models suggests mutant forms of FUS exert an unknown gain-of-toxic function in motor neurons, but mechanisms underlying this effect remain unknown....

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 28273913
DOI: 10.1038/s41598-017-00091-1
PMCID: PMC5428330

Genes

FUS
SOD1

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

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ALS-linked FUS exerts a gain of toxic function involving aberrant p38 MAPK activation.PMID 28273913Read ↗

ALS-associated mutations in FUS disrupt the axonal distribution and function of SMN.Semantic 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 ↗

Expression of human FUS protein in Drosophila leads to progressive neurodegeneration.Semantic proximity Read ↗

Widespread Mislocalization of FUS Is Associated With Mitochondrial Abnormalities in Skeletal Muscle in Amyotrophic Lateral Sclerosis With FUS Mutations.Semantic proximity Read ↗

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

Mutations in FUS lead to synaptic dysregulation in ALS-iPSC derived neurons.Semantic proximity Read ↗

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

Stepwise acquirement of hallmark neuropathology in FUS-ALS iPSC models depends on mutation type and neuronal aging.Semantic proximity Read ↗

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

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

Fused in Sarcoma Neuropathology in Neurodegenerative Disease.Semantic proximity Read ↗

ALS mutations in FUS cause neuronal dysfunction and death in Caenorhabditis elegans by a dominant gain-of-function mechanism.Semantic proximity Read ↗

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