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Article

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function.

Nature communications - 4 Feb 2016

Sharma Aarti, Lyashchenko Alexander K, Lu Lei, Nasrabady Sara Ebrahimi, Elmaleh Margot, Mendelsohn Monica, Nemes Adriana, Tapia Juan Carlos, Mentis George Z, Shneider Neil A

Abstract excerpt

Mutations in FUS cause amyotrophic lateral sclerosis (ALS), including some of the most aggressive, juvenile-onset forms of the disease. FUS loss-of-function and toxic gain-of-function mechanisms have been proposed to explain how mutant FUS leads to motor neuron degeneration, but neither has been firmly established in the pathogenesis of ALS. Here we characterize a series of transgenic FUS mouse lines that...

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Identifiers

PMID: 26842965
DOI: 10.1038/ncomms10465
PMCID: PMC4742863

Genes

FUS

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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ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function.PMID 26842965Read ↗

Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis.Semantic proximity Read ↗

Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss.Semantic proximity Read ↗

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FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration.Semantic proximity Read ↗

Humanized mutant FUS drives progressive motor neuron degeneration without aggregation in ‘FUSDelta14’ knockin miceSemantic proximity Read ↗

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

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

Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice.Semantic 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 ↗

Low level of expression of C-terminally truncated human FUS causes extensive changes in spinal cord transcriptome of asymptomatic transgenic miceSemantic proximity Read ↗

Activity-dependent FUS dysregulation disrupts synaptic homeostasisSemantic proximity Read ↗

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