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Cited by 668

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 access
Warrick, John M, Paulson, Henry L, Gray-Board, Gladys L, Bui, Quang T, Fischbeck, Kenneth H, Pittman, Randall N, Bonini, Nancy M·1998·Cell

Abstract

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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Crossref · 10.1016/s0092-8674(00)81200-3

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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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CoChat. (2026). Citation check: Expanded Polyglutamine Protein Forms Nuclear Inclusions and Causes Neural Degeneration in Drosophila. https://app.cochat.ai/papers/10.1016/s0092-8674(00)81200-3
The paper
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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