The DOI matches its Crossref record; 201 of the 202 references it cites were found in a registry or an index.
Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis
Open accessAbstract
Amyotrophic lateral sclerosis (ALS) is a genetically diverse disease. At least 15 ALS-associated gene loci have so far been identified, and the causative gene is known in approximately 30% of familial ALS cases. Less is known about the factors underlying the sporadic form of the disease. The molecular mechanisms of motor neuron degeneration are best understood in the subtype of disease caused by mutations in superoxide dismutase 1, with a current consensus that motor neuron injury is caused by a complex interplay between multiple pathogenic processes. A key recent finding is that mutated TAR DNA-binding protein 43 is a major constituent of the ubiquitinated protein inclusions in ALS, providing a possible link between the genetic mutation and the cellular pathology. New insights have also indicated the importance of dysregulated glial cell-motor neuron crosstalk, and have highlighted the vulnerability of the distal axonal compartment early in the disease course. In addition, recent studies have suggested that disordered RNA processing is likely to represent a major contributing factor to motor neuron disease. Ongoing research on the cellular pathways highlighted in this Review is predicted to open the door to new therapeutic interventions to slow disease progression in ALS.
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“Approximately 10% of ALS cases are familial with autosomal dominant inheritance of mutations reported in at least 15 genes, including to name a few SOD1 , TARDBP , FUS , ANG , and OPTN (11, 18, 174, 465), and an expanded hexanucleotide repeat of GGGGCC in a noncoding region of the C9ORF72 gene (140, 497).”
From Blood-Brain Barrier: From Physiology to Disease and Back (2019) · cites this paper as [174]
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2 retracted
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A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD
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Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6
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Oussini, H. E. (2016) · theses.fr (ABES) · doi:10.70675/2431e8ddzd4e4z4710z81dcz14100daa6294
Picchiarelli, G. (2018) · theses.fr (ABES) · doi:10.70675/0252f66fz38f9z46b6z8f3ez97b2f3467520 · Cited by 1
D'amico, D. (2020) · theses.fr (ABES) · doi:10.70675/6e54c5b3zdabfz4677z9604zfcce72b7ed92
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Gall, L. L. (2019) · theses.fr (ABES) · doi:10.70675/61e48a01z5133z45e6z8569z1b35b4b8fb4f
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Zhou, L., Li, M., Dai, Q., Liu, X., Li, C., Jiao, H., Pan, H., & Xu, R. (2026) · CNS Neuroscience & Therapeutics · doi:10.1002/cns.70946
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Shahsavari, K., Yazarlu, O., Ahmadnia, H., Ardakani, M. T., Khanavi, M., Esfahani, H. M., Abdollahzadeh, R., sajedi, A., Kazemizadeh, H., & Hasanpour, M. (2026) · Molecular Biology Reports · doi:10.1007/s11033-026-12300-0
Ciuro, M., Rovetto, C., Sirna, A. A., Giunta, S., Leanza, G., & Gulino, R. (2026) · Biology · doi:10.3390/biology15161334
Dresel, F. C., Michaelis, M., & Gourlay, C. W. (2026) · International Journal of Cell Biology · doi:10.1155/ijcb/3022967
Maksimovic, K., Rambabu, M., Santos, J. R., Chan, C., Zelaya, A., Lee, J., Dias, M., Gluscencova, O. B., Youssef, M., Kim, S., et al. (2026) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.64898/2026.08.26.747343
Dissecting cross-talk between microglia and motoneurons in ALS: signaling events and soluble factors
Barbosa, M. (2013) · Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT)
Metadata
- Authors
- Ferraiuolo, L., Kirby, J., Grierson, A. J., Sendtner, M., & Shaw, P. J.
- Affiliations
- University of Sheffield (GB)
- University of Würzburg (DE)
- Corresponding author
- Shaw, Pamela J.
- Venue
- Nature Reviews Neurology
- Published
- 2011-11
- Volume
- 7
- Issue
- 11
- Pages
- 616-630
- ISSN
- 1759-4758 (ISSN-L)
- 1759-4766
- OpenAlex
- W2025413109
- Cited by
- 615
- References
- 202
- Citations by year
- 2026: 12 · 2025: 24 · 2024: 34 · 2023: 25 · 2022: 27 · 2021: 54 · 2020: 34 · 2019: 37 · 2018: 33 · 2017: 42 · 2016: 58 · 2015: 63 · 2014: 63 · 2013: 64 · 2012: 45
- FWCI
- 6.83
- Type
- journal article
- Language
- en
- Open access
- green
- zenodo.org
- Licence
- other-oa
- www.springer.com/tdm (tdm)
- Locations
- Nature Reviews Neurology (publishedVersion)
- PubMed (publishedVersion)
- Zenodo (CERN European Organization for Nuclear Research) (submittedVersion, other-oa)
- Funders
- Wellcome Trust
- Topics
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
- Keywords
- amyotrophic lateral sclerosis, motor neuron injury, motor neuron degeneration, oxidative stress, mitochondrial dysfunction, excitotoxicity, superoxide dismutase 1, TDP-43, sporadic ALS, familial ALS, therapeutic target
- MeSH terms
- Amyotrophic Lateral Sclerosis, Humans, Motor Neurons, Nerve Degeneration
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