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Article

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

Acta neuropathologica - 1 Jun 2017

Scekic-Zahirovic Jelena, Oussini Hajer El, Mersmann Sina, Drenner Kevin, Wagner Marina, Sun Ying, Allmeroth Kira, Dieterlé Stéphane, Sinniger Jérôme, Dirrig-Grosch Sylvie, René Frédérique, Dormann Dorothee, Haass Christian, Ludolph Albert C, Lagier-Tourenne Clotilde, Storkebaum Erik, Dupuis Luc

Abstract excerpt

Motor neuron-extrinsic mechanisms have been shown to participate in the pathogenesis of ALS-SOD1, one familial form of amyotrophic lateral sclerosis (ALS). It remains unclear whether such mechanisms contribute to other familial forms, such as TDP-43 and FUS-associated ALS. Here, we characterize a single-copy mouse model of ALS-FUS that conditionally expresses a disease-relevant truncating FUS mutant from the...

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Identifiers

PMID: 28243725
DOI: 10.1007/s00401-017-1687-9
PMCID: PMC5427169

Genes

FUS
SOD1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026.

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Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis.PMID 28243725Read ↗

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