The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Caspase Cleavage of Gene Products Associated with Triplet Expansion Disorders Generates Truncated Fragments Containing the Polyglutamine Tract
Open accessAbstract
The neurodegenerative diseases Huntington disease, dentatorubropallidoluysian atrophy, spinocerebellar atrophy type 3, and spinal bulbar muscular atrophy are caused by expansion of a polyglutamine tract within their respective gene products. There is increasing evidence that generation of truncated proteins containing an expanded polyglutamine tract may be a key step in the pathogenesis of these disorders. We now report that, similar to huntingtin, atrophin-1, ataxin-3, and the androgen receptor are cleaved in apoptotic extracts. Furthermore, each of these proteins is cleaved by one or more purified caspases, cysteine proteases involved in apoptotic death. The CAG length does not modulate susceptibility to cleavage of any of the full-length proteins. Our results suggest that by generation of truncated polyglutamine-containing proteins, caspase cleavage may represent a common step in the pathogenesis of each of these neurodegenerative diseases.
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Cites
Genetic control of programmed cell death in the nematode C. elegans
Ellis, H. (1986) · Cell · doi:10.1016/0092-8674(86)90004-8 · Cited by 1,828
Cited by
Mammalian Caspases: Structure, Activation, Substrates, and Functions During Apoptosis
Earnshaw, W. C., Martins, L. M., & Kaufmann, S. H. (1999) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.68.1.383 · Cited by 2,760
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
Arrasate, M., Mitra, S., Schweitzer, E. S., Segal, M. R., & Finkbeiner, S. (2004) · Nature · doi:10.1038/nature02998 · Cited by 1,979
The roles of intracellular protein-degradation pathways in neurodegeneration
Rubinsztein, D. C. (2006) · Nature · doi:10.1038/nature05291 · Cited by 1,625
Caspase structure, proteolytic substrates, and function during apoptotic cell death
Nicholson, D. (1999) · Cell Death & Differentiation · doi:10.1038/sj.cdd.4400598 · Cited by 1,543
Self-citationGenin, M., Clement, F., Fattaccioli, A., Raes, M., & Michiels, C. (2015) · BMC Cancer · doi:10.1186/s12885-015-1546-9 · Cited by 1,090
Metadata
- Authors
- Wellington, C. L., Ellerby, L. M., Hackam, A. S., Margolis, R. L., Trifiro, M. A., Singaraja, R., McCutcheon, K., Salvesen, G. S., Propp, S. S., Bromm, M., Rowland, K. J., Zhang, T., Rasper, D., Roy, S., Thornberry, N., Pinsky, L., Kakizuka, A., Ross, C. A., Nicholson, D. W., Bredesen, D. E., & Hayden, M. R.
- Venue
- Journal of Biological Chemistry
- Publisher
- Elsevier BV
- Published
- 1998-04
- Volume
- 273
- Issue
- 15
- Pages
- 9158-9167
- ISSN
- 0021-9258 (ISSN-L)
- OpenAlex
- W1988110501
- Cited by
- 562
- References
- 1
- FWCI
- 19.18
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- The paper
- Wellington, C. L., Ellerby, L. M., Hackam, A. S., Margolis, R. L., Trifiro, M. A., Singaraja, R., McCutcheon, K., Salvesen, G. S., Propp, S. S., Bromm, M., Rowland, K. J., Zhang, T., Rasper, D., Roy, S., Thornberry, N., Pinsky, L., Kakizuka, A., Ross, C. A., Nicholson, D. W., . . . Hayden, M. R. (1998). Caspase Cleavage of Gene Products Associated with Triplet Expansion Disorders Generates Truncated Fragments Containing the Polyglutamine Tract. Journal of Biological Chemistry. https://doi.org/10.1074/jbc.273.15.9158
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
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.
