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Updated: Jan 29, 2026
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Evaluation of Motor Impairment in C. elegans Models of Amyotrophic Lateral Sclerosis
Published on: September 2, 2021
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Rab-dependent cellular trafficking and amyotrophic lateral sclerosis.
S Parakh1,2, E R Perri1,2, C J Jagaraj1
1a Faculty of Medicine and Health Sciences, Department of Biomedical Sciences, Centre for MND Research , Macquarie University , Sydney , Australia.
Critical Reviews in Biochemistry and Molecular Biology
|February 12, 2019
Summary
Rab GTPases are crucial for intracellular transport, and their dysfunction is increasingly linked to neurodegenerative diseases like amyotrophic lateral sclerosis (ALS). This review focuses on Rab GTPase roles in ALS pathophysiology.
Area of Science:
- Neuroscience
- Cell Biology
Background:
- Amyotrophic lateral sclerosis (ALS) involves intracellular transport defects, particularly in motor neurons.
- The role of Rab GTPases in ALS pathogenesis has been historically overlooked.
- Axonal transport defects are well-studied, but cell body transport and Rab GTPase involvement are less understood.
Purpose of the Study:
- To review current understanding of cellular transport defects in ALS.
- To highlight the specific role of Rab GTPases in ALS pathophysiology.
- To explore molecular mechanisms of Rab protein dysfunction in ALS.
Main Methods:
- Literature review and synthesis of recent findings.
- Focus on Rab GTPase function in intracellular transport.
- Discussion of emerging transport-related mechanisms in ALS.
Main Results:
- Rab GTPase dysfunction is increasingly implicated in ALS.
- C9orf72's role in intracellular transport involves Rab GTPases.
- Dysfunctional Rab proteins may specifically target neurons in ALS.
Conclusions:
- Rab GTPase dysfunction is a significant, underappreciated factor in ALS.
- Understanding Rab GTPase roles is key to elucidating ALS mechanisms.
- Further research into nucleocytoplasmic transport and MAMs may reveal additional Rab GTPase involvement in ALS.
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