The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila
Abstract
At least eight dominant human neurodegenerative diseases are due to the expansion of a polyglutamine within the disease proteins. This confers toxicity on the proteins and is associated with nuclear inclusion formation. Recent findings indicate that molecular chaperones can modulate polyglutamine pathogenesis, but the basis of polyglutamine toxicity and the mechanism by which chaperones suppress neurodegeneration remains unknown. In a Drosophila: disease model, we demonstrate that chaperones show substrate specificity for polyglutamine protein, as well as synergy in suppression of neurotoxicity. Our analysis also reveals that chaperones alter the solubility properties of the protein, indicating that chaperone modulation of neurodegeneration in vivo is associated with altered biochemical properties of the mutant polyglutamine protein. These findings have implications for these and other human neurodegenerative diseases associated with abnormal protein aggregation.
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Cites
Scherzinger, E., Lurz, R., Turmaine, M., Mangiarini, L., Hollenbach, B., Hasenbank, R., Bates, G. P., Davies, S. W., Lehrach, H., & Wanker, E. E. (1997) · Cell · doi:10.1016/s0092-8674(00)80514-0 · Cited by 1,291 · cited 1×
“glutamine protein in flies, as in mice and human cells (20,21),”
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Cited by
Yoshida, H. (2007) · The FEBS Journal · doi:10.1111/j.1742-4658.2007.05639.x · Cited by 1,086
Cummings, C. J. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.14.1511 · Cited by 485
Cellular Toxicity of Polyglutamine Expansion Proteins
Schaffar, G., Breuer, P., Boteva, R., Behrends, C., Tzvetkov, N., Strippel, N., Sakahira, H., Siegers, K., Hayer-Hartl, M., & Hartl, F. U. (2004) · Molecular Cell · doi:10.1016/j.molcel.2004.06.029 · Cited by 425
Drosophila as a Model for Human Neurodegenerative Disease
Bilen, J., & Bonini, N. M. (2005) · Annual Review of Genetics · doi:10.1146/annurev.genet.39.110304.095804 · Cited by 422
Therapeutic potential of mood stabilizers lithium and valproic acid:Beyond bipolar disorder
Chiu, C.-T., Wang, Z., Hunsberger, J. G., & Chuang, D.-M. (2013) · Pharmacological Reviews · doi:10.1124/pr.111.005512 · Cited by 404
Metadata
- Authors
- Chan, H. Y. E.
- Venue
- Human Molecular Genetics
- Publisher
- Oxford University Press (OUP)
- Published
- 2000-11-01
- Volume
- 9
- Issue
- 19
- Pages
- 2811-2820
- ISSN
- 0964-6906 (ISSN-L)
- 1460-2083
- OpenAlex
- W2170594193
- Cited by
- 322
- References
- 1
- FWCI
- 9.57
- Type
- journal article
- Language
- en
- Open access
- closed
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- Chan, H. Y. E. (2000). Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila. Human Molecular Genetics. https://doi.org/10.1093/hmg/9.19.2811
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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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