The DOI matches its Crossref record; 26 of the 26 references it cites were found in a registry or an index.
Necroptosis Drives Motor Neuron Death in Models of Both Sporadic and Familial ALS
Open accessAbstract
Most cases of neurodegenerative diseases are sporadic, hindering the use of genetic mouse models to analyze disease mechanisms. Focusing on the motor neuron (MN) disease amyotrophic lateral sclerosis (ALS), we therefore devised a fully humanized coculture model composed of human adult primary sporadic ALS (sALS) astrocytes and human embryonic stem-cell-derived MNs. The model reproduces the cardinal features of human ALS: sALS astrocytes, but not those from control patients, trigger selective death of MNs. The mechanisms underlying this non-cell-autonomous toxicity were investigated in both astrocytes and MNs. Although causal in familial ALS (fALS), SOD1 does not contribute to the toxicity of sALS astrocytes. Death of MNs triggered by either sALS or fALS astrocytes occurs through necroptosis, a form of programmed necrosis involving receptor-interacting protein 1 and the mixed lineage kinase domain-like protein. The necroptotic pathway therefore constitutes a potential therapeutic target for this incurable disease.
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Cites
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Cited by
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Necroptosis and RIPK1-mediated neuroinflammation in CNS diseases
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Necroptosis: a regulated inflammatory mode of cell death
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RIPK1 mediates axonal degeneration by promoting inflammation and necroptosis in ALS
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Necroptosis activation in Alzheimer's disease
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Metadata
- Authors
- Re, D. B., Le Verche, V., Yu, C., Amoroso, M. W., Politi, K. A., Phani, S., Ikiz, B., Hoffmann, L., Koolen, M., Nagata, T., Papadimitriou, D., Nagy, P., Mitsumoto, H., Kariya, S., Wichterle, H., Henderson, C. E., & Przedborski, S.
- Venue
- Neuron
- Publisher
- Elsevier BV
- Published
- 2014-03
- Volume
- 81
- Issue
- 5
- Pages
- 1001-1008
- ISSN
- 0896-6273 (ISSN-L)
- OpenAlex
- W2030194678
- Cited by
- 406
- References
- 26
- FWCI
- 28.52
- Type
- journal article
- Language
- en
- Open access
- hybrid
- Licence
- www.elsevier.com/tdm/userlicense/1.0/ (tdm)
- www.elsevier.com/open-access/userlicense/1.0/ (vor, 369 d)
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- The paper
- Re, D. B., Le Verche, V., Yu, C., Amoroso, M. W., Politi, K. A., Phani, S., Ikiz, B., Hoffmann, L., Koolen, M., Nagata, T., Papadimitriou, D., Nagy, P., Mitsumoto, H., Kariya, S., Wichterle, H., Henderson, C. E., & Przedborski, S. (2014). Necroptosis Drives Motor Neuron Death in Models of Both Sporadic and Familial ALS. Neuron. https://doi.org/10.1016/j.neuron.2014.01.011
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
