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Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases.
Open accessAbstract
Four inherited neurodegenerative diseases are linked to abnormally expanded repeats of glutamine residues in the affected proteins. Molecular modeling followed by optical, electron, and x-ray diffraction studies of a synthetic poly(L-glutamine) shows that it forms beta-sheets strongly held together by hydrogen bonds. Glutamine repeats may function as polar zippers, for example, by joining specific transcription factors bound to separate DNA segments. Their extension may cause disease either by increased, nonspecific affinity between such factors or by gradual precipitation of the affected proteins in neurons.
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Cited by
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DiFiglia, M., Sapp, E., Chase, K. O., Davies, S. W., Bates, G. P., Vonsattel, J. P., & Aronin, N. (1997) · Science · doi:10.1126/science.277.5334.1990 · Cited by 2,439
Unfolding the role of protein misfolding in neurodegenerative diseases
Soto, C. (2003) · Nature Reviews Neuroscience · doi:10.1038/nrn1007 · Cited by 1,506
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
Toxic Proteins in Neurodegenerative Disease
Taylor, J. P., Hardy, J., & Fischbeck, K. H. (2002) · Science · doi:10.1126/science.1067122 · Cited by 1,251
Nuclear and Neuropil Aggregates in Huntington’s Disease: Relationship to Neuropathology
Gutekunst, C.-A., Li, S.-H., Yi, H., Mulroy, J. S., Kuemmerle, S., Jones, R., Rye, D., Ferrante, R. J., Hersch, S. M., & Li, X.-J. (1999) · The Journal of Neuroscience · doi:10.1523/jneurosci.19-07-02522.1999 · Cited by 914
Metadata
- Authors
- Perutz, M. F., Johnson, T., Suzuki, M., & Finch, J. T.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 1994-06-07
- Volume
- 91
- Issue
- 12
- Pages
- 5355-5358
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W2032255653
- Cited by
- 1,062
- References
- 0
- FWCI
- 11.10
- Type
- journal article
- Language
- en
- Open access
- green
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- CoChat. (2026). Citation check: Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases. https://app.cochat.ai/papers/10.1073/pnas.91.12.5355
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- Perutz, M. F., Johnson, T., Suzuki, M., & Finch, J. T. (1994). Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.91.12.5355
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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.
