The Complex Molecular Biology of Amyotrophic Lateral Sclerosis (ALS)
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Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder that causes selective death of motor neurons followed by paralysis and death. A subset of ALS cases is caused by mutations in the gene for Cu, Zn superoxide dismutase (SOD1), which impart a toxic gain of function to this antioxidant enzyme. This neurotoxic property is widely believed to stem from an increased propensity to misfold and aggregate caused by decreased stability of the native homodimer or a tendency to lose stabilizing posttranslational modifications. Study of the molecular mechanisms of SOD1-related ALS has revealed a complex array of interconnected pathological processes, including glutamate excitotoxicity, dysregulation of neurotrophic factors and axon guidance proteins, axonal transport defects, mitochondrial dysfunction, deficient protein quality control, and aberrant RNA processing. Many of these pathologies are directly exacerbated by misfolded and aggregated SOD1 and/or cytosolic calcium overload, suggesting the primacy of these events in disease etiology and their potential as targets for therapeutic intervention.
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With global expression of germline mutations associated with ALS, neural and non-neural tissues manifest the detrimental effects of compromised autophagy, but to varying extents.
“with global expression of germline mutations associated with ALS, neural and non-neural tissues manifest the detrimental effects of compromised autophagy [11], but to varying extents [365,366]”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [366]
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Toxic SOD1 trimers are off-pathway in the formation of amyloid-like fibrils in ALS
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Diseases of the nervous system
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Modulating the Folding Landscape of Superoxide Dismutase 1 with Targeted Molecular Binders
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- Authors
- Redler, R. L., & Dokholyan, N. V.
- Affiliations
- University of North Carolina at Chapel Hill (US)
- Venue
- Progress in Molecular Biology and Translational Science
- Publisher
- Elsevier
- Published
- 2012
- Volume
- 107
- Pages
- 215-262
- ISSN
- 1877-1173 (ISSN-L)
- PMC
- PMC3605887
- OpenAlex
- W57985618
- Cited by
- 172
- References
- 387
- Citations by year
- 2026: 4 · 2025: 5 · 2024: 10 · 2023: 12 · 2022: 11 · 2021: 11 · 2020: 6 · 2019: 12 · 2018: 22 · 2017: 14 · 2016: 18 · 2015: 8 · 2014: 18 · 2013: 18 · 2012: 2
- FWCI
- 37.72
- Type
- book chapter
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Progress in molecular biology and translational science (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institute of General Medical Sciences (R01GM080742)
- National Institute of Neurological Disorders and Stroke (F31NS073435)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Keywords
- amyotrophic lateral sclerosis, SOD1 mutations, Cu/Zn superoxide dismutase, protein misfolding, protein aggregation, glutamate excitotoxicity, neurotrophic factors, mitochondrial dysfunction, protein quality control, RNA processing, calcium overload, motor neuron degeneration, neurodegeneration
- MeSH terms
- Superoxide Dismutase-1, Amyotrophic Lateral Sclerosis, Animals, Axons, Humans, Models, Molecular, Motor Neurons, Necrosis, Nerve Growth Factors, RNA Processing, Post-Transcriptional, Superoxide Dismutase, Apoptosis, Protein Folding, Mice, Protein Multimerization, Translational Research, Biomedical
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
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