The DOI matches its Crossref record; 5 of the 5 references it cites were found in a registry or an index.
Senataxin, Defective in the Neurodegenerative Disorder Ataxia with Oculomotor Apraxia 2, Lies at the Interface of Transcription and the DNA Damage Response
Open accessAbstract
The neurodegenerative disorder ataxia with oculomotor apraxia 2 (AOA-2) is caused by defects in senataxin, a putative RNA/DNA helicase thought to be involved in the termination of transcription at RNA polymerase pause sites. RNA/DNA hybrids (R loops) that arise during transcription pausing lead to genome instability unless they are resolved efficiently. We found that senataxin forms distinct nuclear foci in S/G(2)-phase human cells and that the number of these foci increases in response to impaired DNA replication or DNA damage. Senataxin colocalizes with 53BP1, a key DNA damage response protein, and with other factors involved in DNA repair. Inhibition of transcription using α-amanitin, or the dissolution of R loops by transient expression of RNase H1, leads to the loss of senataxin foci. These results indicate that senataxin localizes to sites of collision between components of the replisome and the transcription apparatus and that it is targeted to R loops, where it plays an important role at the interface of transcription and the DNA damage response.
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Cites
Global analysis of protein expression in yeast
Ghaemmaghami, S., Huh, W.-K., Bower, K., Howson, R. W., Belle, A., Dephoure, N., O'Shea, E. K., & Weissman, J. S. (2003) · Nature · doi:10.1038/nature02046 · Cited by 3,750
Activation of the DNA damage checkpoint and genomic instability in human precancerous lesions
Gorgoulis, V. G., Vassiliou, L.-V. F., Karakaidos, P., Zacharatos, P., Kotsinas, A., Liloglou, T., Venere, M., DiTullio, R. A., Kastrinakis, N. G., Levy, B., et al. (2005) · Nature · doi:10.1038/nature03485 · Cited by 2,009
Defective DNA Repair and Neurodegenerative Disease
Rass, U., Ahel, I., & West, S. C. (2007) · Cell · doi:10.1016/j.cell.2007.08.043 · Cited by 358
Self-citationDNA repair deficiency and neurological disease
McKinnon, P. J. (2009) · Nature Reviews Neuroscience · doi:10.1038/nrn2559 · Cited by 323
Down-regulation of WW Domain-containing Oxidoreductase Induces Tau Phosphorylation in Vitro
Sze, C.-I., Su, M., Pugazhenthi, S., Jambal, P., Hsu, L.-J., Heath, J., Schultz, L., & Chang, N.-S. (2004) · Journal of Biological Chemistry · doi:10.1074/jbc.m401399200 · Cited by 130
Cited by
Causes and consequences of replication stress
Zeman, M. K., & Cimprich, K. A. (2014) · Nature Cell Biology · doi:10.1038/ncb2897 · Cited by 2,079
R-Loops as Cellular Regulators and Genomic Threats
Crossley, M. P., Bocek, M., & Cimprich, K. A. (2019) · Molecular Cell · doi:10.1016/j.molcel.2019.01.024 · Cited by 746
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Regulation of long non-coding RNAs and genome dynamics by the RNA surveillance machinery
Nair, L., Chung, H., & Basu, U. (2020) · Nature Reviews Molecular Cell Biology · doi:10.1038/s41580-019-0209-0 · Cited by 203
Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1
Barmada, S. J., Ju, S., Arjun, A., Batarse, A., Archbold, H. C., Peisach, D., Li, X., Zhang, Y., Tank, E. M. H., Qiu, H., et al. (2015) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1509744112 · Cited by 125
Metadata
- Authors
- Yüce, Ö., & West, S. C.
- Venue
- Molecular and Cellular Biology
- Publisher
- Informa UK Limited
- Published
- 2013-01-01
- Volume
- 33
- Issue
- 2
- Pages
- 406-417
- ISSN
- 0270-7306 (ISSN-L)
- 1098-5549
- OpenAlex
- W2120588059
- Cited by
- 191
- References
- 5
- FWCI
- 5.69
- Type
- journal article
- Language
- en
- Open access
- bronze
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- CoChat. (2026). Citation check: Senataxin, Defective in the Neurodegenerative Disorder Ataxia with Oculomotor Apraxia 2, Lies at the Interface of Transcription and the DNA Damage Response. https://app.cochat.ai/papers/10.1128/mcb.01195-12
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- Yüce, Ö., & West, S. C. (2013). Senataxin, Defective in the Neurodegenerative Disorder Ataxia with Oculomotor Apraxia 2, Lies at the Interface of Transcription and the DNA Damage Response. Molecular and Cellular Biology. https://doi.org/10.1128/mcb.01195-12
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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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