The DOI matches its Crossref record; 2 of the 2 references it cites were found in a registry or an index.
Polyglutamine-Expanded Androgen Receptors Form Aggregates That Sequester Heat Shock Proteins, Proteasome Components and SRC-1, and Are Suppressed by the HDJ-2 Chaperone
Abstract
Spinal bulbar muscular atrophy is a neurodegenerative disorder caused by a polyglutamine expansion in the androgen receptor (AR). We show in transiently transfected HeLa cells that an AR containing 48 glutamines (ARQ48) accumulates in a hormone-dependent manner in both cytoplasmic and nuclear aggregates. Electron microscopy reveals both types of aggregates to have a similar ultrastructure. ARQ48 aggregates sequester mitochondria and steroid receptor coactivator 1 and stain positively for NEDD8, Hsp70, Hsp90 and HDJ-2/HSDJ. Co-expression of HDJ-2/HSDJ significantly represses aggregate formation. ARQ48 aggregates also label with antibodies recognizing the PA700 proteasome caps but not 20S core particles. These results suggest that ARQ48 accumulates due to protein misfolding and a breakdown in proteolytic processing. Furthermore, the homeostatic disturbances associated with aggregate formation may affect normal cell function.
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Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1
Orr, H. T., Chung, M.-Y., Banfi, S., Kwiatkowski, T. J., Servadio, A., Beaudet, A. L., McCall, A. E., Duvick, L. A., Ranum, L. P. W., & Zoghbi, H. Y. (1993) · Nature Genetics · doi:10.1038/ng0793-221 · Cited by 1,632
Veldscholte, J., Berrevoets, C. A., Zegers, N. D., Van der Kwast, T. H., Grootegoed, J. A., & Mulder, E. (1992) · Biochemistry · doi:10.1021/bi00147a029 · Cited by 139
Cited by
Jana, N. R. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.13.2009 · Cited by 432
Serine 776 of Ataxin-1 Is Critical for Polyglutamine-Induced Disease in SCA1 Transgenic Mice
Emamian, E. S., Kaytor, M. D., Duvick, L. A., Zu, T., Tousey, S. K., Zoghbi, H. Y., Clark, H. B., & Orr, H. T. (2003) · Neuron · doi:10.1016/s0896-6273(03)00258-7 · Cited by 305
The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies
Bauer, P. O., & Nukina, N. (2009) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2009.06302.x · Cited by 178
Identification of ter94, Drosophila VCP, as a modulator of polyglutamine-induced neurodegeneration
Higashiyama, H., Hirose, F., Yamaguchi, M., Inoue, Y. H., Fujikake, N., Matsukage, A., & Kakizuka, A. (2002) · Cell Death & Differentiation · doi:10.1038/sj.cdd.4400955 · Cited by 129
Eftekharzadeh, B., Banduseela, V. C., Chiesa, G., Martínez-Cristóbal, P., Rauch, J. N., Nath, S. R., Schwarz, D. M. C., Shao, H., Marin-Argany, M., Di Sanza, C., et al. (2019) · Nature Communications · doi:10.1038/s41467-019-11594-y · Cited by 76
Metadata
- Authors
- Stenoien, D. L., Cummings, C. J., Adams, H. P., Mancini, M. G., Patel, K., DeMartino, G. N., Marcelli, M., Weigel, N. L., & Mancini, M. A.
- Venue
- Human Molecular Genetics
- Publisher
- Oxford University Press (OUP)
- Published
- 1999-05-01
- Volume
- 8
- Issue
- 5
- Pages
- 731-741
- ISSN
- 0964-6906 (ISSN-L)
- 1460-2083
- OpenAlex
- W2165589182
- Cited by
- 444
- References
- 2
- FWCI
- 15.12
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: Polyglutamine-Expanded Androgen Receptors Form Aggregates That Sequester Heat Shock Proteins, Proteasome Components and SRC-1, and Are Suppressed by the HDJ-2 Chaperone. https://app.cochat.ai/papers/10.1093/hmg/8.5.731
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- Stenoien, D. L., Cummings, C. J., Adams, H. P., Mancini, M. G., Patel, K., DeMartino, G. N., Marcelli, M., Weigel, N. L., & Mancini, M. A. (1999). Polyglutamine-Expanded Androgen Receptors Form Aggregates That Sequester Heat Shock Proteins, Proteasome Components and SRC-1, and Are Suppressed by the HDJ-2 Chaperone. Human Molecular Genetics. https://doi.org/10.1093/hmg/8.5.731
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
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.
