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Cited by 371

The DOI matches its Crossref record; 53 of the 53 references it cites were found in a registry or an index.

Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans

Open access
Satyal, Sanjeev H., Schmidt, Enrico, Kitagawa, Kazunori, Sondheimer, Neal, Lindquist, Susan, Kramer, James M., Morimoto, Richard I.·2000·Proceedings of the National Academy of Sciences

Abstract

Expansion of polyglutamine repeats in several unrelated proteins causes neurodegenerative diseases with distinct but related pathologies. To provide a model system for investigating common pathogenic features, we have examined the behavior of polyglutamine expansions expressed in Caenorhabditis elegans . The expression of polyglutamine repeats as green fluorescent protein (GFP)-fusion proteins in body wall muscle cells causes discrete cytoplasmic aggregates that appear early in embryogenesis and correlates with a delay in larval to adult development. The heat shock response is activated idiosyncratically in individual cells in a polyglutamine length-dependent fashion. The toxic effect of polyglutamine expression and the formation of aggregates can be reversed by coexpression of the yeast chaperone Hsp104. The altered homeostasis associated with polyglutamine aggregates causes both the sequestration of an otherwise soluble protein with shorter arrays of glutamine repeats and the relocalization of a nuclear glutamine-rich protein. These observations of induced aggregation and relocalization have implications for disorders involving protein aggregation.

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Metadata

Authors
Satyal, S. H., Schmidt, E., Kitagawa, K., Sondheimer, N., Lindquist, S., Kramer, J. M., & Morimoto, R. I.
Venue
Proceedings of the National Academy of Sciences
Published
2000-05-16
Volume
97
Issue
11
Pages
5750-5755
ISSN
  • 0027-8424 (ISSN-L)
  • 1091-6490
OpenAlex
W2169579807
Cited by
371
References
53
FWCI
8.95
Type
journal article
Language
en
Open access
green

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CoChat. (2026). Citation check: Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans. https://app.cochat.ai/papers/10.1073/pnas.100107297
The paper
Satyal, S. H., Schmidt, E., Kitagawa, K., Sondheimer, N., Lindquist, S., Kramer, J. M., & Morimoto, R. I. (2000). Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.100107297

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