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Related Experiment Video

Updated: Feb 12, 2026

Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells
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Stress Granule Assembly Disrupts Nucleocytoplasmic Transport.

Ke Zhang1, J Gavin Daigle1, Kathleen M Cunningham2

  • 1Department of Neurology, School of Medicine, Johns Hopkins University, Baltimore, MD 21205, USA; Brain Science Institute, School of Medicine, Johns Hopkins University, Baltimore, MD 21205, USA.

Cell
|April 10, 2018
PubMed
Summary

Cellular stress disrupts nucleocytoplasmic transport in neurodegenerative diseases like ALS and FTD by forming stress granules. Inhibiting these granules, for example, by targeting Ataxin-2, can prevent transport defects and neurodegeneration.

Keywords:
ALSC9ORF72nucleocytoplasmic transportstress granule

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Area of Science:

  • Neuroscience
  • Molecular Biology
  • Genetics

Background:

  • Nucleocytoplasmic transport defects are key in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), often linked to C9ORF72 repeat expansions.
  • Protein aggregation in other neurodegenerative diseases also suggests disrupted nucleocytoplasmic transport as a shared pathogenic mechanism.

Purpose of the Study:

  • To investigate how cellular stress impacts nucleocytoplasmic transport in the context of C9ORF72-mediated ALS/FTD.
  • To identify molecular links between stress granules and nucleocytoplasmic transport defects in neurodegeneration.

Main Methods:

  • Examined the localization of nucleocytoplasmic transport factors under cellular stress conditions.
  • Investigated the role of stress granules in nucleocytoplasmic transport disruption.
  • Assessed the therapeutic potential of inhibiting stress granule assembly (e.g., via Ataxin-2 knockdown) in a C9ORF72-ALS/FTD model.

Main Results:

  • Cellular stress causes critical nucleocytoplasmic transport factors to accumulate in stress granules.
  • Stress granules are directly implicated in disrupting nucleocytoplasmic transport.
  • Inhibiting stress granule formation, specifically by reducing Ataxin-2 levels, ameliorates nucleocytoplasmic transport defects and neurodegeneration in C9ORF72-ALS/FTD.

Conclusions:

  • Established a direct link between stress granule assembly and nucleocytoplasmic transport dysfunction in C9ORF72-mediated ALS/FTD.
  • Findings highlight stress granules as a critical therapeutic target for ALS/FTD and potentially other neurodegenerative diseases involving protein stress.
  • Demonstrated that targeting Ataxin-2 can mitigate key pathological features of C9ORF72-ALS/FTD.