The DOI matches its Crossref record; 7 of the 7 references it cites were found in a registry or an index.
Nuclear localization sequence of FUS and induction of stress granules by ALS mutants
Open accessAbstract
Mutations in FUS have been reported to cause a subset of familial amyotrophic lateral sclerosis (ALS) cases. Wild-type FUS is mostly localized in the nuclei of neurons, but the ALS mutants are partly mislocalized in the cytoplasm and can form inclusions. Little is known about the regulation of FUS subcellular localization or how the ALS mutations alter FUS function. Here we demonstrate that the C-terminal 32 amino acid residues of FUS constitute an effective nuclear localization sequence (NLS) as it targeted beta-galactosidase (LacZ, 116 kDa) to the nucleus. Deletion of or the ALS point mutations within the NLS caused cytoplasmic mislocalization of FUS. Moreover, we identified the poly-A binding protein (PABP1), a stress granule marker, as an interacting partner of FUS. PABP1 formed large cytoplasmic foci that co-localized with the mutant FUS inclusions. No such foci, which resemble stress granules, were observed in the presence of wild-type FUS. In addition, processing bodies, which are functionally related to stress granules, were adjacent to but not co-localized with the mutant FUS inclusions. Our results suggest that the ALS mutations in the C-terminal NLS of FUS can impair FUS nuclear localization and induce cytoplasmic mislocalization, inclusion formation, and potential perturbation of RNA metabolism.
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Ubiquitinated 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
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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
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
Kabashi, E., Valdmanis, P. N., Dion, P., Spiegelman, D., McConkey, B. J., Velde, C. V., Bouchard, J.-P., Lacomblez, L., Pochigaeva, K., Salachas, F., et al. (2008) · Nature Genetics · doi:10.1038/ng.132 · Cited by 1,544
TLS (FUS) binds RNA in vivo and engages in nucleo-cytoplasmic shuttling
Zinszner, H., Sok, J., Immanuel, D., Yin, Y., & Ron, D. (1997) · Journal of Cell Science · doi:10.1242/jcs.110.15.1741 · Cited by 350
Sequestosome 1/p62 links familial ALS mutant SOD1 to LC3 via an ubiquitin‐independent mechanism
Gal, J., Ström, A., Kwinter, D. M., Kilty, R., Zhang, J., Shi, P., Fu, W., Wooten, M. W., & Zhu, H. (2009) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2009.06388.x · Cited by 187
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Han, T. W., Kato, M., Xie, S., Wu, L. C., Mirzaei, H., Pei, J., Chen, M., Xie, Y., Allen, J., Xiao, G., et al. (2012) · Cell · doi:10.1016/j.cell.2012.04.016 · Cited by 767
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Metadata
- Authors
- Gal, J., Zhang, J., Kwinter, D. M., Zhai, J., Jia, H., Jia, J., & Zhu, H.
- Venue
- Neurobiology of Aging
- Publisher
- Elsevier BV
- Published
- 2011-12
- Volume
- 32
- Issue
- 12
- Pages
- 2323.e27-2323.e40
- ISSN
- 0197-4580 (ISSN-L)
- OpenAlex
- W2086896046
- Cited by
- 215
- References
- 7
- FWCI
- 12.33
- Type
- journal article
- Language
- en
- Open access
- green
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- Gal, J., Zhang, J., Kwinter, D. M., Zhai, J., Jia, H., Jia, J., & Zhu, H. (2011). Nuclear localization sequence of FUS and induction of stress granules by ALS mutants. Neurobiology of Aging. https://doi.org/10.1016/j.neurobiolaging.2010.06.010
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