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Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology
Open accessAbstract
Cu, Zn superoxide dismutase (SOD1) has been detected within spinal cord mitochondria of mutant SOD1 transgenic mice, a model of familial ALS. The copper chaperone for SOD1 (CCS) provides SOD1 with copper, facilitates the conversion of immature apo-SOD1 to a mature holoform, and influences in yeast the cytosolic/mitochondrial partitioning of SOD1. To determine how CCS affects G93A-SOD1-induced disease, we generated transgenic mice overexpressing CCS and crossed them to G93A-SOD1 or wild-type SOD1 transgenic mice. Both CCS transgenic mice and CCS/wild-type-SOD1 dual transgenic mice are neurologically normal. In contrast, CCS/G93A-SOD1 dual transgenic mice develop accelerated neurological deficits, with a mean survival of 36 days, compared with 242 days for G93A-SOD1 mice. Immuno-EM and subcellular fractionation studies on the spinal cord show that G93A-SOD1 is enriched within mitochondria in the presence of CCS overexpression. Our results indicate that CCS overexpression in G93A-SOD1 mice produces severe mitochondrial pathology and accelerates disease course.
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Urushitani, M., Sik, A., Sakurai, T., Nukina, N., Takahashi, R., & Julien, J.-P. (2006) · Nature Neuroscience · doi:10.1038/nn1603 · Cited by 419
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Metadata
- Authors
- Son, M., Puttaparthi, K., Kawamata, H., Rajendran, B., Boyer, P. J., Manfredi, G., & Elliott, J. L.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 2007-04-03
- Volume
- 104
- Issue
- 14
- Pages
- 6072-6077
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W2062900509
- Cited by
- 161
- References
- 1
- FWCI
- 12.08
- Type
- journal article
- Language
- en
- Open access
- green
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- Son, M., Puttaparthi, K., Kawamata, H., Rajendran, B., Boyer, P. J., Manfredi, G., & Elliott, J. L. (2007). Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.0610923104
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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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