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Article

U1 snRNP is mislocalized in ALS patient fibroblasts bearing NLS mutations in FUS and is required for motor neuron outgrowth in zebrafish.

Nucleic acids research - 31 Mar 2015

Yu Yong, Chi Binkai, Xia Wei, Gangopadhyay Jaya, Yamazaki Tomohiro, Winkelbauer-Hurt Marlene E, Yin Shanye, Eliasse Yoan, Adams Edward, Shaw Christopher E, Reed Robin

Abstract excerpt

Mutations in FUS cause amyotrophic lateral sclerosis (ALS), but the molecular pathways leading to neurodegeneration remain obscure. We previously found that U1 snRNP is the most abundant FUS interactor. Here, we report that components of the U1 snRNP core particle (Sm proteins and U1 snRNA), but not the mature U1 snRNP-specific proteins (U1-70K, U1A and U1C), co-mislocalize with FUS to the cytoplasm in ALS...

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Share to Amyotrophic Lateral Sclerosis
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Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Share to Motor Neurons

Share to Nuclear Localization Signals

Share to Protein Interaction Domains and Motifs

Share to Ribonucleoprotein, U1 Small Nuclear

Share to RNA-Binding Protein FUS

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Source

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Identifiers

PMID: 25735748
DOI: 10.1093/nar/gkv157
PMCID: PMC4381066

Genes

FUS

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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U1 snRNP is mislocalized in ALS patient fibroblasts bearing NLS mutations in FUS and is required for motor neuron outgrowth in zebrafish.PMID 25735748Read ↗

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