The DOI matches its Crossref record; 7 of the 7 references it cites were found in a registry or an index.
Lifespan Extension and Rescue of Spongiform Encephalopathy in Superoxide Dismutase 2 Nullizygous Mice Treated with Superoxide Dismutase–Catalase Mimetics
Open accessAbstract
Superoxide is produced as a result of normal energy metabolism within the mitochondria and is scavenged by the mitochondrial form of superoxide dismutase (sod2). Mice with inactivated SOD2 (sod2 nullizygous mice) die prematurely, exhibiting several metabolic and mitochondrial defects and severe tissue pathologies, including a lethal spongiform neurodegenerative disorder (Li et al., 1995; Melov et al., 1998, 1999). We show that treatment of sod2 nullizygous mice with synthetic superoxide dismutase (SOD)-catalase mimetics extends their lifespan by threefold, rescues the spongiform encephalopathy, and attenuates mitochondrial defects. This class of antioxidant compounds has been shown previously to extend lifespan in the nematode Caenorhabditis elegans (Melov et al., 2000). These new findings in mice suggest novel therapeutic approaches to neurodegenerative diseases associated with oxidative stress such as Friedreich ataxia, spongiform encephalopathies, and Alzheimer's and Parkinson's diseases, in which chronic oxidative damage to the brain has been implicated.
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Cites
Dilated cardiomyopathy and neonatal lethality in mutant mice lacking manganese superoxide dismutase
Li, Y., Huang, T.-T., Carlson, E. J., Melov, S., Ursell, P. C., Olson, J. L., Noble, L. J., Yoshimura, M. P., Berger, C., Chan, P. H., et al. (1995) · Nature Genetics · doi:10.1038/ng1295-376 · Cited by 1,789
Self-citationCherny, R. A., Atwood, C. S., Xilinas, M. E., Gray, D. N., Jones, W. D., McLean, C. A., Barnham, K. J., Volitakis, I., Fraser, F. W., Kim, Y.-S., et al. (2001) · Neuron · doi:10.1016/s0896-6273(01)00317-8 · Cited by 1,447
Praticò, D., Uryu, K., Leight, S., Trojanoswki, J. Q., & Lee, V. M.-Y. (2001) · The Journal of Neuroscience · doi:10.1523/jneurosci.21-12-04183.2001 · Cited by 777
Puccio, H., Simon, D., Cossée, M., Criqui-Filipe, P., Tiziano, F., Melki, J., Hindelang, C., Matyas, R., Rustin, P., & Koenig, M. (2001) · Nature Genetics · doi:10.1038/84818 · Cited by 738
Dependence of H2O2 formation by rat heart mitochondria on substrate availability and donor age
Hansford, R. G., Hogue, B. A., & Mildaziene, V. (1997) · Journal of Bioenergetics and Biomembranes · doi:10.1023/a:1022420007908 · Cited by 447 · cited 2×
“4–4% of all oxygen consumed during normal respiration is converted into superoxide within the mitochondrion (Chance et al., 1979; Turrens and Boveris, 1980; Boveris, 1984; Turrens et al., 1985; Hansford et al., 1997), the chief source of reactive oxygen species (ROS) within the cell.”
BackgroundInfluentialClinical, biochemical and molecular genetic correlations in Friedreich's ataxia
Bradley, J. L. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.2.275 · Cited by 339
Jama, J. W., Launer, L. J., Witteman, J. C. M., den Breeijen, J. H., Breteler, M. M. B., Grobbee, D. E., & Hofman, A. (1996) · American Journal of Epidemiology · doi:10.1093/oxfordjournals.aje.a008922 · Cited by 252
Cited by
Wallace, D. C. (2005) · Annual Review of Genetics · doi:10.1146/annurev.genet.39.110304.095751 · Cited by 3,185
Self-citationNOX enzymes and the biology of reactive oxygen
Lambeth, J. D. (2004) · Nature Reviews Immunology · doi:10.1038/nri1312 · Cited by 3,125
Oxidative stress and cancer: Have we moved forward?
Halliwell, B. (2007) · Biochemical Journal · doi:10.1042/bj20061131 · Cited by 1,302
Role of Catalase in Oxidative Stress- and Age-Associated Degenerative Diseases
Nandi, A., Yan, L.-J., Jana, C. K., & Das, N. (2019) · Oxidative Medicine and Cellular Longevity · doi:10.1155/2019/9613090 · Cited by 1,185
Mitochondria and reactive oxygen species
Kowaltowski, A. J., de Souza-Pinto, N. C., Castilho, R. F., & Vercesi, A. E. (2009) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2009.05.004 · Cited by 1,162
Metadata
- Authors
- Melov, S., Doctrow, S. R., Schneider, J. A., Haberson, J., Patel, M., Coskun, P. E., Huffman, K., Wallace, D. C., & Malfroy, B.
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 2001-11-01
- Volume
- 21
- Issue
- 21
- Pages
- 8348-8353
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- OpenAlex
- W2170632631
- Cited by
- 277
- References
- 7
- FWCI
- 4.44
- Type
- journal article
- Language
- en
- Open access
- bronze
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- CoChat. (2026). Citation check: Lifespan Extension and Rescue of Spongiform Encephalopathy in Superoxide Dismutase 2 Nullizygous Mice Treated with Superoxide Dismutase–Catalase Mimetics. https://app.cochat.ai/papers/10.1523/jneurosci.21-21-08348.2001
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- Melov, S., Doctrow, S. R., Schneider, J. A., Haberson, J., Patel, M., Coskun, P. E., Huffman, K., Wallace, D. C., & Malfroy, B. (2001). Lifespan Extension and Rescue of Spongiform Encephalopathy in Superoxide Dismutase 2 Nullizygous Mice Treated with Superoxide Dismutase–Catalase Mimetics. The Journal of Neuroscience. https://doi.org/10.1523/jneurosci.21-21-08348.2001
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