Journal article
New perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis
Translational Neurodegeneration, Vol.10(1), Art. 46
2021
Abstract
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by selective, early degeneration of motor neurons in the brain and spinal cord. Motor neurons have long axonal projections, which rely on the integrity of neuronal cytoskeleton and mitochondria to regulate energy requirements for maintaining axonal stability, anterograde and retrograde transport, and signaling between neurons. The formation of protein aggregates which contain cytoskeletal proteins, and mitochondrial dysfunction both have devastating effects on the function of neurons and are shared pathological features across several neurodegenerative conditions, including ALS, Alzheimer's disease, Parkinson's disease, Huntington’s disease and Charcot-Marie-Tooth disease. Furthermore, it is becoming increasingly clear that cytoskeletal integrity and mitochondrial function are intricately linked. Therefore, dysregulations of the cytoskeletal network and mitochondrial homeostasis and localization, may be common pathways in the initial steps of neurodegeneration. Here we review and discuss known contributors, including variants in genetic loci and aberrant protein activities, which modify cytoskeletal integrity, axonal transport and mitochondrial localization in ALS and have overlapping features with other neurodegenerative diseases. Additionally, we explore some emerging pathways that may contribute to this disruption in ALS.
Details
- Title
- New perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis
- Authors/Creators
- F. Theunissen (Author/Creator) - Murdoch UniversityP.K. West (Author/Creator) - Genomics (United Kingdom)S. Brennan (Author/Creator) - Genomics (United Kingdom)B. Petrović (Author/Creator) - The University of SydneyK. Hooshmand (Author/Creator) - The University of SydneyP.A. Akkari (Author/Creator) - Murdoch UniversityM. Keon (Author/Creator) - Genomics (United Kingdom)B. Guennewig (Author/Creator) - The University of Sydney
- Publication Details
- Translational Neurodegeneration, Vol.10(1), Art. 46
- Publisher
- BioMed Central Ltd as part of Springer Nature
- Identifiers
- 991005543043007891
- Copyright
- © 2021 The Authors.
- Murdoch Affiliation
- Centre for Molecular Medicine and Innovative Therapeutics
- Language
- English
- Resource Type
- Journal article
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- Collaboration types
- Domestic collaboration
- International collaboration
- Citation topics
- 1 Clinical & Life Sciences
- 1.52 Neurodegenerative Diseases
- 1.52.765 ALS Mechanisms
- Web Of Science research areas
- Neurosciences
- ESI research areas
- Neuroscience & Behavior