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

doi:10.15252/embj.201592559

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Article

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

The EMBO journal - 17 May 2016

Scekic-Zahirovic Jelena, Sendscheid Oliver, El Oussini Hajer, Jambeau Mélanie, Sun Ying, Mersmann Sina, Wagner Marina, Dieterlé Stéphane, Sinniger Jérome, Dirrig-Grosch Sylvie, Drenner Kevin, Birling Marie-Christine, Qiu Jinsong, Zhou Yu, Li Hairi, Fu Xiang-Dong, Rouaux Caroline, Shelkovnikova Tatyana, Witting Anke, Ludolph Albert C, Kiefer Friedemann, Storkebaum Erik, Lagier-Tourenne Clotilde, Dupuis Luc

Abstract excerpt

FUS is an RNA-binding protein involved in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Cytoplasmic FUS-containing aggregates are often associated with concomitant loss of nuclear FUS Whether loss of nuclear FUS function, gain of a cytoplasmic function, or a combination of both lead to neurodegeneration remains elusive. To address this question, we generated knockin mice expressing...

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PMID: 26951610
DOI: 10.15252/embj.201592559
PMCID: PMC4868956

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Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss.PMID 26951610Read ↗

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