The DOI matches its Crossref record; 27 of the 27 references it cites were found in a registry or an index.
The Influence of Huntingtin Protein Size on Nuclear Localization and Cellular Toxicity
Open accessAbstract
Huntington disease is an autosomal dominant neurodegenerative disorder caused by the pathological expansion of a polyglutamine tract. In this study we directly assess the influence of protein size on the formation and subcellular localization of huntingtin aggregates. We have created numerous deletion constructs expressing successively smaller fragments of huntingtin and show that these smaller proteins containing 128 glutamines form both intranuclear and perinuclear aggregates. In contrast, larger NH2-terminal fragments of huntingtin proteins with 128 glutamines form exclusively perinuclear aggregates. These aggregates can form in the absence of endogenous huntingtin. Furthermore, expression of mutant huntingtin results in increased susceptibility to apoptotic stress that is greater with decreasing protein length and increasing polyglutamine size. As both intranuclear and perinuclear aggregates are clearly associated with increased cellular toxicity, this supports an important role for toxic polyglutamine-containing fragments forming aggregates and playing a key role in the pathogenesis of Huntington disease.
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Cites
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Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain
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Nuclear targeting sequences — a consensus?
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“This also supports the in vitro data generated by assessing interaction between glutathione-S-transferase fusion proteins with expanded polyglutamine tracts (Scherzinger et al., 1997).”
ResultGörlich, D., & Mattaj, I. W. (1996) · Science · doi:10.1126/science.271.5255.1513 · Cited by 1,086
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Self-citationIntranuclear Inclusions of Expanded Polyglutamine Protein in Spinocerebellar Ataxia Type 3
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Cited by
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
Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease
Zuccato, C., Valenza, M., & Cattaneo, E. (2010) · Physiological Reviews · doi:10.1152/physrev.00041.2009 · Cited by 848
Huntingtin Expression Stimulates Endosomal–Lysosomal Activity, Endosome Tubulation, and Autophagy
Kegel, K. B., Kim, M., Sapp, E., McIntyre, C., Castaño, J. G., Aronin, N., & DiFiglia, M. (2000) · The Journal of Neuroscience · doi:10.1523/jneurosci.20-19-07268.2000 · Cited by 533
p53 Mediates Cellular Dysfunction and Behavioral Abnormalities in Huntington’s Disease
Bae, B.-I., Xu, H., Igarashi, S., Fujimuro, M., Agrawal, N., Taya, Y., Hayward, S. D., Moran, T. H., Montell, C., Ross, C. A., et al. (2005) · Neuron · doi:10.1016/j.neuron.2005.06.005 · Cited by 485
Huntington's disease: from pathology and genetics to potential therapies
Imarisio, S., Carmichael, J., Korolchuk, V., Chen, C.-W., Saiki, S., Rose, C., Krishna, G., Davies, J. E., Ttofi, E., Underwood, B. R., et al. (2008) · Biochemical Journal · doi:10.1042/bj20071619 · Cited by 409
Metadata
- Authors
- Hackam, A. S., Singaraja, R., Wellington, C. L., Metzler, M., McCutcheon, K., Zhang, T., Kalchman, M., & Hayden, M. R.
- Venue
- The Journal of Cell Biology
- Publisher
- Rockefeller University Press
- Published
- 1998-06-01
- Volume
- 141
- Issue
- 5
- Pages
- 1097-1105
- ISSN
- 0021-9525 (ISSN-L)
- 1540-8140
- OpenAlex
- W2107792324
- Cited by
- 313
- References
- 27
- FWCI
- 9.00
- Type
- journal article
- Language
- en
- Open access
- green
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- Hackam, A. S., Singaraja, R., Wellington, C. L., Metzler, M., McCutcheon, K., Zhang, T., Kalchman, M., & Hayden, M. R. (1998). The Influence of Huntingtin Protein Size on Nuclear Localization and Cellular Toxicity. The Journal of Cell Biology. https://doi.org/10.1083/jcb.141.5.1097
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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