The DOI matches its Crossref record; 39 of the 39 references it cites were found in a registry or an index.
Expanded Polyglutamine Protein Forms Nuclear Inclusions and Causes Neural Degeneration in Drosophila
Open accessAbstract
Spinocerebellar ataxia type 3 (SCA3/MJD) is one of at least eight human neurodegenerative diseases caused by glutamine-repeat expansion. We have recreated glutamine-repeat disease in Drosophila using a segment of the SCA3/MJD protein. Targeted expression of the protein with an expanded polyglutamine repeat led to nuclear inclusion (NI) formation and late-onset cell degeneration. Differential sensitivity to the mutant transgene was observed among different cell types, with neurons being particularly susceptible; NI formation alone was not sufficient for degeneration. The viral antiapoptotic gene P35 mitigated polyglutamine-induced degeneration in vivo. Our results demonstrate that cellular mechanisms of human glutamine-repeat disease are conserved in invertebrates. This fly model will aid in identifying additional factors that modulate neurodegeneration.
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Metadata
- Authors
- Warrick, J. M., Paulson, H. L., Gray-Board, G. L., Bui, Q. T., Fischbeck, K. H., Pittman, R. N., & Bonini, N. M.
- Venue
- Cell
- Publisher
- Elsevier BV
- Published
- 1998-06
- Volume
- 93
- Issue
- 6
- Pages
- 939-949
- ISSN
- 0092-8674 (ISSN-L)
- OpenAlex
- W2135124405
- Cited by
- 668
- References
- 39
- FWCI
- 16.57
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Warrick, J. M., Paulson, H. L., Gray-Board, G. L., Bui, Q. T., Fischbeck, K. H., Pittman, R. N., & Bonini, N. M. (1998). Expanded Polyglutamine Protein Forms Nuclear Inclusions and Causes Neural Degeneration in Drosophila. Cell. https://doi.org/10.1016/s0092-8674(00)81200-3
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