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Updated: Jan 24, 2026
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Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells
Published on: May 16, 2017
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Nucleocytoplasmic transport defects in neurodegeneration - Cause or consequence?
Saskia Hutten1, Dorothee Dormann2
1BioMedical Center (BMC), Ludwig-Maximilians-University Munich, 82152 Planegg-Martinsried, Germany.
Seminars in Cell & Developmental Biology
|June 2, 2019
Summary
Defects in nucleocytoplasmic transport are linked to neurodegenerative diseases like ALS and Alzheimer's. This review explores how transport issues contribute to protein aggregate formation and disease progression.
Area of Science:
- Neurobiology
- Molecular Biology
- Cell Biology
Background:
- Nucleocytoplasmic transport is crucial for cellular function.
- Defects in this transport are implicated in neurodegenerative diseases.
- The causal relationship between transport defects and protein aggregates remains unclear.
Purpose of the Study:
- To review alterations in nucleocytoplasmic transport machinery in neurodegenerative diseases.
- To discuss the functional impact of these transport defects.
- To explore parallels with aging and progeria for mechanistic insights.
Main Methods:
- Literature review of recent findings.
- Analysis of alterations in soluble and stationary transport components.
- Comparison with physiological aging and progeria.
Main Results:
- Nucleocytoplasmic transport defects are observed in amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), Alzheimer's disease (AD), and Huntington's disease (HD).
- These defects affect the transport of proteins and mRNAs.
- Similarities exist between transport alterations in neurodegeneration, aging, and progeria.
Conclusions:
- Impaired nucleocytoplasmic transport is a significant factor in neurodegenerative disease pathogenesis.
- Understanding these transport defects may offer new therapeutic strategies.
- Insights from aging and progeria can illuminate neurodegenerative mechanisms.
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