The DOI matches its Crossref record; 6 of the 6 references it cites were found in a registry or an index.
A Yeast Model of FUS/TLS-Dependent Cytotoxicity
Open accessAbstract
FUS/TLS is a nucleic acid binding protein that, when mutated, can cause a subset of familial amyotrophic lateral sclerosis (fALS). Although FUS/TLS is normally located predominantly in the nucleus, the pathogenic mutant forms of FUS/TLS traffic to, and form inclusions in, the cytoplasm of affected spinal motor neurons or glia. Here we report a yeast model of human FUS/TLS expression that recapitulates multiple salient features of the pathology of the disease-causing mutant proteins, including nuclear to cytoplasmic translocation, inclusion formation, and cytotoxicity. Protein domain analysis indicates that the carboxyl-terminus of FUS/TLS, where most of the ALS-associated mutations are clustered, is required but not sufficient for the toxicity of the protein. A genome-wide genetic screen using a yeast over-expression library identified five yeast DNA/RNA binding proteins, encoded by the yeast genes ECM32, NAM8, SBP1, SKO1, and VHR1, that rescue the toxicity of human FUS/TLS without changing its expression level, cytoplasmic translocation, or inclusion formation. Furthermore, hUPF1, a human homologue of ECM32, also rescues the toxicity of FUS/TLS in this model, validating the yeast model and implicating a possible insufficiency in RNA processing or the RNA quality control machinery in the mechanism of FUS/TLS mediated toxicity. Examination of the effect of FUS/TLS expression on the decay of selected mRNAs in yeast indicates that the nonsense-mediated decay pathway is probably not the major determinant of either toxicity or suppression.
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Rosen, D. R., Siddique, T., Patterson, D., Figlewicz, D. A., Sapp, P., Hentati, A., Donaldson, D., Goto, J., O'Regan, J. P., Deng, H.-X., et al. (1993) · Nature · doi:10.1038/362059a0 · Cited by 7,062
Self-citationUbiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
Neumann, M., Sampathu, D. M., Kwong, L. K., Truax, A. C., Micsenyi, M. C., Chou, T. T., Bruce, J., Schuck, T., Grossman, M., Clark, C. M., et al. (2006) · Science · doi:10.1126/science.1134108 · Cited by 6,576
TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
Sreedharan, J., Blair, I. P., Tripathi, V. B., Hu, X., Vance, C., Rogelj, B., Ackerley, S., Durnall, J. C., Williams, K. L., Buratti, E., et al. (2008) · Science · doi:10.1126/science.1154584 · Cited by 2,630
Mutations of optineurin in amyotrophic lateral sclerosis
Maruyama, H., Morino, H., Ito, H., Izumi, Y., Kato, H., Watanabe, Y., Kinoshita, Y., Kamada, M., Nodera, H., Suzuki, H., et al. (2010) · Nature · doi:10.1038/nature08971 · Cited by 1,265
Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
Ilieva, H., Polymenidou, M., & Cleveland, D. W. (2009) · Journal of Cell Biology · doi:10.1083/jcb.200908164 · Cited by 1,008
Yeast Genes That Enhance the Toxicity of a Mutant Huntingtin Fragment or α-Synuclein
Willingham, S., Outeiro, T. F., DeVit, M. J., Lindquist, S. L., & Muchowski, P. J. (2003) · Science · doi:10.1126/science.1090389 · Cited by 422
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Cited by
Monahan, Z., Ryan, V. H., Janke, A. M., Burke, K. A., Rhoads, S. N., Zerze, G. H., O'Meally, R., Dignon, G. L., Conicella, A. E., Zheng, W., et al. (2017) · The EMBO Journal · doi:10.15252/embj.201696394 · Cited by 684
Protein aggregation in amyotrophic lateral sclerosis
Blokhuis, A. M., Groen, E. J. N., Koppers, M., van den Berg, L. H., & Pasterkamp, R. J. (2013) · Acta Neuropathologica · doi:10.1007/s00401-013-1125-6 · Cited by 579
Gene Overexpression: Uses, Mechanisms, and Interpretation
Prelich, G. (2012) · Genetics · doi:10.1534/genetics.111.136911 · Cited by 490
Guo, L., Kim, H. J., Wang, H., Monaghan, J., Freyermuth, F., Sung, J. C., O’Donovan, K., Fare, C. M., Diaz, Z., Singh, N., et al. (2018) · Cell · doi:10.1016/j.cell.2018.03.002 · Cited by 466
Sun, Z., Diaz, Z., Fang, X., Hart, M. P., Chesi, A., Shorter, J., & Gitler, A. D. (2011) · PLoS Biology · doi:10.1371/journal.pbio.1000614 · Cited by 458
Metadata
- Authors
- Ju, S., Tardiff, D. F., Han, H., Divya, K., Zhong, Q., Maquat, L. E., Bosco, D. A., Hayward, L. J., Brown, R. H., Lindquist, S., Ringe, D., & Petsko, G. A.
- Venue
- PLoS Biology
- Publisher
- Public Library of Science (PLoS)
- Published
- 2011-04-26
- Volume
- 9
- Issue
- 4
- Pages
- e1001052
- ISSN
- 1544-9173 (ISSN-L)
- 1545-7885
- OpenAlex
- W1599327564
- Cited by
- 207
- References
- 6
- FWCI
- 11.34
- Type
- journal article
- Language
- en
- Open access
- gold
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- Ju, S., Tardiff, D. F., Han, H., Divya, K., Zhong, Q., Maquat, L. E., Bosco, D. A., Hayward, L. J., Brown, R. H., Lindquist, S., Ringe, D., & Petsko, G. A. (2011). A Yeast Model of FUS/TLS-Dependent Cytotoxicity. PLoS Biology. https://doi.org/10.1371/journal.pbio.1001052
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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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