Article

Low level of expression of C-terminally truncated human FUS causes extensive changes in spinal cord transcriptome of asymptomatic transgenic mice

2019-07-03

Abstract excerpt

Mutations in a gene encoding RNA-binding protein FUS was linked to familial forms of amyotrophic lateral sclerosis (ALS). C-terminal truncations of FUS are associated with aggressive forms of ALS. However, motor neurons are able to tolerate permanent production of pathogenic truncated form of FUS protein until its accumulation in the cytoplasm of neurones does not reach a critical threshold. In order to identify h...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: f9df56fb-305d-52ab-b6f6-596313a76dc7
DOI: 10.1101/689414

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Low level of expression of C-terminally truncated human FUS causes extensive changes in spinal cord transcriptome of asymptomatic transgenic miceDOI 10.1101/689414Read

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

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

Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashionSemantic proximity Read

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

Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulationSemantic proximity Read

Activity-dependent FUS dysregulation disrupts synaptic homeostasisSemantic proximity Read

Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axisSemantic proximity Read

Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation.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

Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice.Semantic proximity Read

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

FUS(1-359) transgenic mice as a model of ALS: pathophysiological and molecular aspects of the proteinopathySemantic proximity Read