The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Mutant Superoxide Dismutase-1-Linked Familial Amyotrophic Lateral Sclerosis: Molecular Mechanisms of Neuronal Death and Protection
Open accessAbstract
Mutations in human Cu/Zn superoxide dismutase-1 (SOD) cause ∼20% of cases of familial amyotrophic lateral sclerosis (FALS). We investigated the mechanism of mutant SOD-induced neuronal degeneration by expressing wild-type and mutant SODs in neuronal cells by means of infection with replication-deficient recombinant adenoviruses. Expression of two FALS-related mutant SODs (A4V and V148G) caused death of differentiated PC12 cells, superior cervical ganglion neurons, and hippocampal pyramidal neurons. Cell death included many features typical of apoptosis. Death could be prevented by copper (Cu 2+) chelators, Bcl-2, glutathione, vitamin E, and inhibitors of caspases. Mutant SOD-expressing PC12 cells had higher rates of superoxide (O 2−) production under a variety of conditions. The results support the hypothesis that mutant SOD induced-neurodegeneration is associated with disturbances of neuronal free radical homeostasis.
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Cites
Trophic Interactions Between Astroglial Cells and Hippocampal Neurons in Culture
Banker, G. A. (1980) · Science · doi:10.1126/science.7403847 · Cited by 674
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Neurodegenerative diseases and oxidative stress
Barnham, K. J., Masters, C. L., & Bush, A. I. (2004) · Nature Reviews Drug Discovery · doi:10.1038/nrd1330 · Cited by 3,683
Pasinelli, P., Belford, M. E., Lennon, N., Bacskai, B. J., Hyman, B. T., Trotti, D., & Brown, R. H. (2004) · Neuron · doi:10.1016/j.neuron.2004.06.021 · Cited by 506
Motoneuron Death Triggered by a Specific Pathway Downstream of Fas
Raoul, C., Estévez, A. G., Nishimune, H., Cleveland, D. W., deLapeyrière, O., Henderson, C. E., Haase, G., & Pettmann, B. (2002) · Neuron · doi:10.1016/s0896-6273(02)00905-4 · Cited by 432
Sankowski, R., Mader, S., & Valdés-Ferrer, S. I. (2015) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2015.00028 · Cited by 351
Increased oxidative damage to DNA in ALS patients
Bogdanov, M., Brown, R. H., Matson, W., Smart, R., Hayden, D., O’Donnell, H., Flint Beal, M., & Cudkowicz, M. (2000) · Free Radical Biology and Medicine · doi:10.1016/s0891-5849(00)00349-x · Cited by 334
Metadata
- Authors
- Ghadge, G. D., Lee, J. P., Bindokas, V. P., Jordan, J., Ma, L., Miller, R. J., & Roos, R. P.
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 1997-11-15
- Volume
- 17
- Issue
- 22
- Pages
- 8756-8766
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- OpenAlex
- W2123413118
- Cited by
- 197
- References
- 1
- FWCI
- 14.57
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- CoChat. (2026). Citation check: Mutant Superoxide Dismutase-1-Linked Familial Amyotrophic Lateral Sclerosis: Molecular Mechanisms of Neuronal Death and Protection. https://app.cochat.ai/papers/10.1523/jneurosci.17-22-08756.1997
- The paper
- Ghadge, G. D., Lee, J. P., Bindokas, V. P., Jordan, J., Ma, L., Miller, R. J., & Roos, R. P. (1997). Mutant Superoxide Dismutase-1-Linked Familial Amyotrophic Lateral Sclerosis: Molecular Mechanisms of Neuronal Death and Protection. The Journal of Neuroscience. https://doi.org/10.1523/jneurosci.17-22-08756.1997
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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.
