The DOI matches its Crossref record; 50 of the 50 references it cites were found in a registry or an index.
Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS
Open accessAbstract
Many mutations confer one or more toxic function(s) on copper/zinc superoxide dismutase 1 (SOD1) that impair motor neuron viability and cause familial amyotrophic lateral sclerosis (FALS). Using a conformation-specific antibody that detects misfolded SOD1 (C4F6), we found that oxidized wild-type SOD1 and mutant SOD1 share a conformational epitope that is not present in normal wild-type SOD1. In a subset of human sporadic ALS (SALS) cases, motor neurons in the lumbosacral spinal cord were markedly C4F6 immunoreactive, indicating that an aberrant wild-type SOD1 species was present. Recombinant, oxidized wild-type SOD1 and wild-type SOD1 immunopurified from SALS tissues inhibited kinesin-based fast axonal transport in a manner similar to that of FALS-linked mutant SOD1. Our findings suggest that wild-type SOD1 can be pathogenic in SALS and identify an SOD1-dependent pathogenic mechanism common to FALS and SALS.
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Cites
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Self-citationAxonal Transport Defects in Neurodegenerative Diseases
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Cited by
Guidelines for the use and interpretation of assays for monitoring autophagy
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Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis
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Superoxide dismutases and superoxide reductases
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Astrocytes from familial and sporadic ALS patients are toxic to motor neurons
Haidet-Phillips, A. M., Hester, M. E., Miranda, C. J., Meyer, K., Braun, L., Frakes, A., Song, S., Likhite, S., Murtha, M. J., Foust, K. D., et al. (2011) · Nature Biotechnology · doi:10.1038/nbt.1957 · Cited by 782
Metadata
- Authors
- Bosco, D. A., Morfini, G., Karabacak, N. M., Song, Y., Gros-Louis, F., Pasinelli, P., Goolsby, H., Fontaine, B. A., Lemay, N., McKenna-Yasek, D., Frosch, M. P., Agar, J. N., Julien, J.-P., Brady, S. T., & Brown, R. H.
- Venue
- Nature Neuroscience
- Published
- 2010-10-17
- Volume
- 13
- Issue
- 11
- Pages
- 1396-1403
- ISSN
- 1097-6256 (ISSN-L)
- 1546-1726
- OpenAlex
- W2157076525
- Cited by
- 686
- References
- 50
- FWCI
- 32.30
- Type
- journal article
- Language
- en
- Open access
- green
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- Bosco, D. A., Morfini, G., Karabacak, N. M., Song, Y., Gros-Louis, F., Pasinelli, P., Goolsby, H., Fontaine, B. A., Lemay, N., McKenna-Yasek, D., Frosch, M. P., Agar, J. N., Julien, J.-P., Brady, S. T., & Brown, R. H. (2010). Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS. Nature Neuroscience. https://doi.org/10.1038/nn.2660
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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.
