The DOI matches its Crossref record; 7 of the 7 references it cites were found in a registry or an index.
Minute quantities of misfolded mutant superoxide dismutase‐1 cause amyotrophic lateral sclerosis
Abstract
Mutant forms of superoxide dismutase-1 (SOD1) cause amyotrophic lateral sclerosis (ALS) by an unknown noxious mechanism. Using an antibody against a novel epitope in the G127insTGGG mutation, mutant SOD1 was studied for the first time in spinal cord and brain of an ALS patient. The level was below 0.5% of the SOD1 level in controls. In corresponding transgenic mice the content of mutant SOD1 was also low, although it was enriched in spinal cord and brain compared with other tissues. In the mice the misfolded mutant SOD1 aggregated rapidly and 20% occurred in steady state as detergent-soluble protoaggregates. The misfolded SOD1 and the protoaggregates form, from birth until death, a potentially noxious burden that may induce the motor neuron injury. Detergent-resistant aggregates, as well as inclusions of mutant SOD1 in motor neurons and astrocytes, accumulated in spinal cord ventral horns of the patient and mice with terminal disease. The inclusions and aggregates may serve as terminal markers of long-term assault by misfolded SOD1 and protoaggregates.
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Cites
Superoxide dismutase: Improved assays and an assay applicable to acrylamide gels
Beauchamp, C., & Fridovich, I. (1971) · Analytical Biochemistry · doi:10.1016/0003-2697(71)90370-8 · Cited by 12,892
Walsh, D. M., Klyubin, I., Fadeeva, J. V., Cullen, W. K., Anwyl, R., Wolfe, M. S., Rowan, M. J., & Selkoe, D. J. (2002) · Nature · doi:10.1038/416535a · Cited by 4,291
Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases
Bucciantini, M., Giannoni, E., Chiti, F., Baroni, F., Formigli, L., Zurdo, J., Taddei, N., Ramponi, G., Dobson, C. M., & Stefani, M. (2002) · Nature · doi:10.1038/416507a · Cited by 2,434
Aggresomes: A Cellular Response to Misfolded Proteins
Johnston, J. A., Ward, C. L., & Kopito, R. R. (1998) · The Journal of Cell Biology · doi:10.1083/jcb.143.7.1883 · Cited by 2,148
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
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Lino, M. M., Schneider, C., & Caroni, P. (2002) · The Journal of Neuroscience · doi:10.1523/jneurosci.22-12-04825.2002 · Cited by 424
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
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
Self-citationDeng, H.-X., Shi, Y., Furukawa, Y., Zhai, H., Fu, R., Liu, E., Gorrie, G. H., Khan, M. S., Hung, W.-Y., Bigio, E. H., et al. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0602046103 · Cited by 431
Amyotrophic lateral sclerosis associated with mutations in the CuZn superoxide dismutase gene
Andersen, P. M. (2006) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-996-0008-9 · Cited by 368
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Metadata
- Authors
- Jonsson, P. A., Ernhill, K., Andersen, P. M., Bergemalm, D., Brännström, T., Gredal, O., Nilsson, P., & Marklund, S. L.
- Venue
- Brain
- Publisher
- Oxford University Press (OUP)
- Published
- 2004-01
- Volume
- 127
- Issue
- 1
- Pages
- 73-88
- ISSN
- 0006-8950 (ISSN-L)
- 1460-2156
- OpenAlex
- W2150430904
- Cited by
- 260
- References
- 7
- FWCI
- 7.66
- Type
- journal article
- Language
- en
- Open access
- closed
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- Jonsson, P. A., Ernhill, K., Andersen, P. M., Bergemalm, D., Brännström, T., Gredal, O., Nilsson, P., & Marklund, S. L. (2004). Minute quantities of misfolded mutant superoxide dismutase‐1 cause amyotrophic lateral sclerosis. Brain. https://doi.org/10.1093/brain/awh005
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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