The DOI matches its Crossref record; 2 of the 2 references it cites were found in a registry or an index.
Mutant Cu, Zn Superoxide Dismutase that Causes Motoneuron Degeneration Is Present in Mitochondria in the CNS
Open accessAbstract
Mutations in Cu, Zn superoxide dismutase (SOD1) cause a fraction of amyotrophic lateral sclerosis (ALS), which involves motoneuron degeneration, paralysis, and death. An acquired activity by mutant SOD1 is responsible for the cellular toxicity, but how mutant SOD1 kills motoneurons is unclear. In transgenic mouse models of ALS, mitochondrial degeneration occurs early, before disease onset, raising the question of how mutant SOD1 damages mitochondria. Here we investigate the intracellular localization of SOD1 in the CNS to determine whether SOD1 is present in mitochondria, where it could directly damage this organelle. We show that endogenous mouse SOD1, wild-type human, and mutant human SOD1 (G93A), when expressed as transgenes, are colocalized with mitochondria in spinal cord by immunofluorescence confocal microscopy. By immunoelectron microscopy, we show that SOD1 is present within mitochondria at similar concentrations as in the cytoplasm. Thus SOD1, in addition to being a cytosolic enzyme, is present inside mitochondria in the CNS.
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Bruijn, L. I., Becher, M. W., Lee, M. K., Anderson, K. L., Jenkins, N. A., Copeland, N. G., Sisodia, S. S., Rothstein, J. D., Borchelt, D. R., Price, D. L., et al. (1997) · Neuron · doi:10.1016/s0896-6273(00)80272-x · Cited by 1,341
Copper,zinc superoxide dismutase is primarily a cytosolic protein in human cells.
Crapo, J. D., Oury, T., Rabouille, C., Slot, J. W., & Chang, L. Y. (1992) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.89.21.10405 · Cited by 503
Cited by
ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors
Boillée, S., Vande Velde, C., & Cleveland, D. W. (2006) · Neuron · doi:10.1016/j.neuron.2006.09.018 · Cited by 1,429
UNRAVELING THE MECHANISMS INVOLVED IN MOTOR NEURON DEGENERATION IN ALS
Bruijn, L. I., Miller, T. M., & Cleveland, D. W. (2004) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.27.070203.144244 · Cited by 1,185
Molecular biology of amyotrophic lateral sclerosis: insights from genetics
Pasinelli, P., & Brown, R. H. (2006) · Nature Reviews Neuroscience · doi:10.1038/nrn1971 · Cited by 1,107
The role of mitochondria in amyotrophic lateral sclerosis
Smith, E. F., Shaw, P. J., & De Vos, K. J. (2019) · Neuroscience Letters · doi:10.1016/j.neulet.2017.06.052 · Cited by 559
Toxicity of Familial ALS-Linked SOD1 Mutants from Selective Recruitment to Spinal Mitochondria
Liu, J., Lillo, C., Jonsson, P. A., Velde, C. V., Ward, C. M., Miller, T. M., Subramaniam, J. R., Rothstein, J. D., Marklund, S., Andersen, P. M., et al. (2004) · Neuron · doi:10.1016/j.neuron.2004.06.016 · Cited by 506
Metadata
- Authors
- Higgins, C. M. J., Jung, C., Ding, H., & Xu, Z.
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 2002-03-15
- Volume
- 22
- Issue
- 6
- Pages
- RC215-RC215
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- OpenAlex
- W2135690573
- Cited by
- 239
- References
- 2
- FWCI
- 12.77
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Higgins, C. M. J., Jung, C., Ding, H., & Xu, Z. (2002). Mutant Cu, Zn Superoxide Dismutase that Causes Motoneuron Degeneration Is Present in Mitochondria in the CNS. The Journal of Neuroscience. https://doi.org/10.1523/jneurosci.22-06-j0001.2002
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