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Article

Dendritic Homeostasis Disruption in a Novel Frontotemporal Dementia Mouse Model Expressing Cytoplasmic Fused in Sarcoma.

EBioMedicine - 1 Oct 2017

Shiihashi Gen, Ito Daisuke, Arai Itaru, Kobayashi Yuki, Hayashi Kanehiro, Otsuka Shintaro, Nakajima Kazunori, Yuzaki Michisuke, Itohara Shigeyoshi, Suzuki Norihiro

Abstract excerpt

Cytoplasmic aggregation of fused in sarcoma (FUS) is detected in brain regions affected by amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), which compose the disease spectrum, FUS proteinopathy. To understand the pathomechanism of ALS-FTD-associated FUS, we examined the behavior and cellular properties of an ALS mouse model overexpressing FUS with nuclear localization signal deletion. Mutant...

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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: 28928015
DOI: 10.1016/j.ebiom.2017.09.005
PMCID: PMC5652009

Genes

FUS

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Related research

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Dendritic Homeostasis Disruption in a Novel Frontotemporal Dementia Mouse Model Expressing Cytoplasmic Fused in Sarcoma.PMID 28928015Read ↗

Activity-dependent FUS dysregulation disrupts synaptic homeostasisSemantic proximity Read ↗

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Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic miceSemantic proximity Read ↗

Neuronal dysfunction caused by FUSR521G promotes ALS-associated phenotypes that are attenuated by NF-κB inhibition.Semantic proximity Read ↗

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Cytoplasmic accumulation of FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and defects in inhibitory synapsesSemantic proximity Read ↗

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