The DOI matches its Crossref record; 33 of the 33 references it cites were found in a registry or an index.
Mutant Huntingtin Expression in Clonal Striatal Cells: Dissociation of Inclusion Formation and Neuronal Survival by Caspase Inhibition
Open accessAbstract
Neuronal intranuclear inclusions are found in the brains of patients with Huntington’s disease and form from the polyglutamine-expanded N-terminal region of mutant huntingtin. To explore the properties of inclusions and their involvement in cell death, mouse clonal striatal cells were transiently transfected with truncated and full-length human wild-type and mutant huntingtin cDNAs. Both normal and mutant proteins localized in the cytoplasm, and infrequently, in dispersed and perinuclear vacuoles. Only mutant huntingtin formed nuclear and cytoplasmic inclusions, which increased with polyglutamine expansion and with time after transfection. Nuclear inclusions contained primarily cleaved N-terminal products, whereas cytoplasmic inclusions contained cleaved and larger intact proteins. Cells with wild-type or mutant protein had distinct apoptotic features (membrane blebbing, shrinkage, cellular fragmentation), but those with mutant huntingtin generated the most cell fragments (apoptotic bodies). The caspase inhibitor Z-VAD-FMK significantly increased cell survival but did not diminish nuclear and cytoplasmic inclusions. In contrast, Z-DEVD-FMK significantly reduced nuclear and cytoplasmic inclusions but did not increase survival. A series of N-terminal products was formed from truncated normal and mutant proteins and from full-length mutant huntingtin but not from full-length wild-type huntingtin. One prominent N-terminal product was blocked by Z-VAD-FMK. In summary, the formation of inclusions in clonal striatal cells corresponds to that seen in the HD brain and is separable from events that regulate cell death. N-terminal cleavage may be linked to mutant huntingtin’s role in cell death.
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Citations
Cites
Apoptosis: A Basic Biological Phenomenon with Wideranging Implications in Tissue Kinetics
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Self-citationAggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain
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BackgroundIntranuclear Inclusions of Expanded Polyglutamine Protein in Spinocerebellar Ataxia Type 3
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Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons
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BackgroundMethodSelf-citationEvidence for apoptotic cell death in Huntington disease and excitotoxic animal models
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Cited by
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
Arrasate, M., Mitra, S., Schweitzer, E. S., Segal, M. R., & Finkbeiner, S. (2004) · Nature · doi:10.1038/nature02998 · Cited by 1,979
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Apoptosis in neurodegenerative disorders
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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
Self-citationHuntington’s Disease: Mechanisms of Pathogenesis and Therapeutic Strategies
Jimenez-Sanchez, M., Licitra, F., Underwood, B. R., & Rubinsztein, D. C. (2017) · Cold Spring Harbor Perspectives in Medicine · doi:10.1101/cshperspect.a024240 · Cited by 507
Metadata
- Authors
- Kim, M., Lee, H.-S., LaForet, G., McIntyre, C., Martin, E. J., Chang, P., Kim, T. W., Williams, M., Reddy, P. H., Tagle, D., Boyce, F. M., Won, L., Heller, A., Aronin, N., & DiFiglia, M.
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 1999-02-01
- Volume
- 19
- Issue
- 3
- Pages
- 964-973
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- OpenAlex
- W2149110755
- Cited by
- 305
- References
- 33
- FWCI
- 11.00
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Kim, M., Lee, H.-S., LaForet, G., McIntyre, C., Martin, E. J., Chang, P., Kim, T. W., Williams, M., Reddy, P. H., Tagle, D., Boyce, F. M., Won, L., Heller, A., Aronin, N., & DiFiglia, M. (1999). Mutant Huntingtin Expression in Clonal Striatal Cells: Dissociation of Inclusion Formation and Neuronal Survival by Caspase Inhibition. The Journal of Neuroscience. https://doi.org/10.1523/jneurosci.19-03-00964.1999
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