Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
Open accessAbstract
Selective degeneration and death of one or more classes of neurons is the defining feature of human neurodegenerative disease. Although traditionally viewed as diseases mainly affecting the most vulnerable neurons, in most instances of inherited disease the causative genes are widely—usually ubiquitously—expressed. Focusing on amyotrophic lateral sclerosis (ALS), especially disease caused by dominant mutations in Cu/Zn superoxide dismutase (SOD1), we review here the evidence that it is the convergence of damage developed within multiple cell types, including within neighboring nonneuronal supporting cells, which is crucial to neuronal dysfunction. Damage to a specific set of key partner cells as well as to vulnerable neurons may account for the selective susceptibility of neuronal subtypes in many human neurodegenerative diseases, including Huntington's disease (HD), Parkinson's disease (PD), prion disease, the spinal cerebellar ataxias (SCAs), and Alzheimer's disease (AD).
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Motor neuron injury in SOD1 mutations results from toxic gain-of-function rather than loss of dismutase activity.
“Motor neuron injury in SOD1 mutations results from toxic gain-of-function rather than loss of dismutase activity (275).”
From Blood-Brain Barrier: From Physiology to Disease and Back (2019) · cites this paper as [275]
ALS is a progressive neurodegenerative disease with a strong non-cell autonomous component contributing to motor neuron death.
“ALS is a progressive neurodegenerative disease with a strong non-cell autonomous component contributing to motor neuron death [17].”
From Distinct repertoires of microRNAs present in mouse astrocytes compared to astrocyte-secreted exosomes (2017) · cites this paper as [17]
ALS and other neurodegenerative disease processes are non-cell autonomous.
“ALS and other neurodegenerative disease processes are non-cell autonomous (Ilieva et al., 2009).”
From MicroRNA Profiling Reveals Marker of Motor Neuron Disease in ALS Models (2017) · cites this paper as Ilieva et al., 2009
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- Authors
- Ilieva, H., Polymenidou, M., & Cleveland, D. W.
- Affiliations
- Ludwig Cancer Research (BE)
- Corresponding author
- Cleveland, Don W.
- Venue
- Journal of Cell Biology
- Publisher
- Rockefeller University Press
- Published
- 2009-12-01
- Volume
- 187
- Issue
- 6
- Pages
- 761-772
- ISSN
- 0021-9525 (ISSN-L)
- 1540-8140
- PMC
- PMC2806318
- OpenAlex
- W2146546145
- Cited by
- 1,008
- References
- 12
- Citations by year
- 2026: 18 · 2025: 30 · 2024: 30 · 2023: 46 · 2022: 33 · 2021: 51 · 2020: 46 · 2019: 65 · 2018: 64 · 2017: 69 · 2016: 78 · 2015: 72 · 2014: 81 · 2013: 89 · 2012: 87
- FWCI
- 8.84
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Licence
- cc-by-nc-sa
- Locations
- The Journal of Cell Biology (publishedVersion)
- PubMed Central (publishedVersion, cc-by-nc-sa)
- PubMed (publishedVersion)
- Funders
- National Institute of Neurological Disorders and Stroke (R37NS027036)
- 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 › Genetic Neurodegenerative Diseases
- Keywords
- neurodegenerative diseases, amyotrophic lateral sclerosis, SOD1 mutations, selective neuronal degeneration, glial cells, astrocytes, microglia, Huntington's disease, Parkinson's disease, prion disease, Alzheimer's disease
- MeSH terms
- Superoxide Dismutase-1, Amyotrophic Lateral Sclerosis, Animals, Astrocytes, Axonal Transport, Capillaries, Endoplasmic Reticulum, Humans, Mutation, Nerve Degeneration, Neurons, Stress, Physiological, Superoxides, Superoxide Dismutase, T-Lymphocytes, Cell Death, Microglia, Glutamic Acid
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.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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