Related Experiment Video
Updated: May 16, 2025
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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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Amyotrophic Lateral Sclerosis: Focus on Cytoplasmic Trafficking and Proteostasis
Shrilaxmi Ms1, Saradindu Banerjee1, Santosh R D'Mello2,3
1Center for Molecular Neuroscience, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India.
Molecular Neurobiology
|April 3, 2025
Summary
Amyotrophic lateral sclerosis (ALS) involves motor neuron loss due to protein aggregate buildup. This review highlights endoplasmic reticulum stress, impaired vesicular transport, and axonal transport defects in ALS pathogenesis.
Area of Science:
- Neuroscience
- Cellular Biology
- Genetics
Background:
- Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease.
- ALS involves progressive loss of upper and lower motor neurons.
- While often multifactorial, some ALS cases stem from inherited gene mutations.
Purpose of the Study:
- To review recent advances in understanding ALS pathogenesis.
- Focus on the roles of endoplasmic reticulum (ER) stress, vesicular transport, and axonal transport in motor neuron degeneration.
Main Methods:
- Review of current scientific literature on ALS.
- Analysis of cellular mechanisms contributing to motor neuron loss.
Main Results:
- Misfolded protein aggregate accumulation is toxic to motor neurons.
- These aggregates disrupt ER function and protein transport.
- Impaired axonal transport of RNA, proteins, and organelles (e.g., mitochondria) contributes to neurodegeneration.
Conclusions:
- Defective ER stress, impaired ER-Golgi vesicular transport, and compromised axonal transport are key contributors to ALS.
- Understanding these cellular dysfunctions is crucial for developing therapeutic strategies for ALS.
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