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Article

Importance of Functional Loss of FUS in FTLD/ALS

3 May 2018

Abstract excerpt

Fused in sarcoma (FUS) is an RNA binding protein that regulates RNA metabolism including alternative splicing, transcription, and RNA transportation. FUS is genetically and pathologically involved in frontotemporal lobar degeneration (FTLD)/ amyotrophic lateral sclerosis (ALS). Multiple lines of evidence across diverse models suggest that functional loss of FUS can lead to neuronal dysfunction and/or neuronal...

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Identifiers

PMID: 29774215
DOI: 10.3389/fmolb.2018.00044

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Related research

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Importance of Functional Loss of FUS in FTLD/ALSPMID 29774215Read ↗

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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 ↗

Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis.Semantic proximity Read ↗

Activity-dependent FUS dysregulation disrupts synaptic homeostasisSemantic proximity Read ↗

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FUS regulates AMPA receptor function and FTLD/ALS-associated behaviour via GluA1 mRNA stabilizationSemantic proximity Read ↗

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