Article

FUS controls muscle differentiation and structure through LLPS mediated recruitment of MEF2 and ETV5

2024-09-19

Abstract excerpt

FUS is an RNA binding protein mutated in amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by progressive muscle weakness. We show that ALS-associated FUS mutations lead to ultrastructural defects in muscle of FUS- ALS patients, with disruption of sarcomeres and mitochondria. Studies in mouse and Drosophila models demonstrate an evolutionary-conserved cell autonomous function of FUS...

Topics

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

Identifiers and source

Literature Corpus work: 031b0607-3acd-5b2d-8e35-137b0b659849
DOI: 10.1101/2024.09.18.613669

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

FUS controls muscle differentiation and structure through LLPS mediated recruitment of MEF2 and ETV5DOI 10.1101/2024.09.18.613669Read

Mutant FUS and ELAVL4 (HuD) Aberrant Crosstalk in Amyotrophic Lateral SclerosisSemantic proximity Read

FUS-ALS mutants alter FMRP phase separation equilibrium and impair protein translation.Semantic proximity Read

FUS-ALS mutants alter FMRP phase separation equilibrium and impair protein translationSemantic proximity Read

Characterization of FUS mutations in amyotrophic lateral sclerosis using RNA-Seq.Semantic proximity Read

Early mitochondrial gene dysregulation precedes motor neuron degeneration in genomically humanised FUS mutant miceSemantic proximity Read

Early mitochondrial gene dysregulation precedes motor neuron degeneration in genomically humanised FUS mutant miceSemantic proximity Read

Synaptic accumulation of FUS triggers age-dependent misregulation of inhibitory synapses in ALS-FUS miceSemantic proximity Read

Synaptic FUS accumulation triggers early misregulation of synaptic RNAs in a mouse model of ALSSemantic proximity Read

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

FUS Mutant Human Motoneurons Display Altered Transcriptome and microRNA Pathways with Implications for ALS Pathogenesis.Semantic proximity Read

Mutation of the ALS-/FTD-Associated RNA-Binding Protein FUS Affects Axonal Development.Semantic proximity Read

FUS P525L mutation causing amyotrophic lateral sclerosis and movement disorders.Semantic proximity Read