The DOI matches its Crossref record; 64 of the 64 references it cites were found in a registry or an index.
Mammalian SUN Protein Interaction Networks at the Inner Nuclear Membrane and Their Role in Laminopathy Disease Processes
Open accessAbstract
The nuclear envelope (NE) LINC complex, in mammals comprised of SUN domain and nesprin proteins, provides a direct connection between the nuclear lamina and the cytoskeleton, which contributes to nuclear positioning and cellular rigidity. SUN1 and SUN2 interact with lamin A, but lamin A is only required for NE localization of SUN2, and it remains unclear how SUN1 is anchored. Here, we identify emerin and short nesprin-2 isoforms as novel nucleoplasmic binding partners of SUN1/2. These have overlapping binding sites distinct from the lamin A binding site. However, we demonstrate that tight association of SUN1 with the nuclear lamina depends upon a short motif within residues 209-228, a region that does not interact significantly with known SUN1 binding partners. Moreover, SUN1 localizes correctly in cells lacking emerin. Importantly then, the major determinant of SUN1 NE localization has yet to be identified. We further find that a subset of lamin A mutations, associated with laminopathies Emery-Dreifuss muscular dystrophy (EDMD) and Hutchinson-Gilford progeria syndrome (HGPS), disrupt lamin A interaction with SUN1 and SUN2. Despite this, NE localization of SUN1 and SUN2 is not impaired in cell lines from either class of patients. Intriguingly, SUN1 expression at the NE is instead enhanced in a significant proportion of HGPS but not EDMD cells and strongly correlates with pre-lamin A accumulation due to preferential interaction of SUN1 with pre-lamin A. We propose that these different perturbations in lamin A-SUN protein interactions may underlie the opposing effects of EDMD and HGPS mutations on nuclear and cellular mechanics.
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Cites
Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome
Eriksson, M., Brown, W. T., Gordon, L. B., Glynn, M. W., Singer, J., Scott, L., Erdos, M. R., Robbins, C. M., Moses, T. Y., Berglund, P., et al. (2003) · Nature · doi:10.1038/nature01629 · Cited by 1,990
Coupling of the nucleus and cytoplasm: Role of the LINC complex
Crisp, M., Liu, Q., Roux, K., Rattner, J. B., Shanahan, C., Burke, B., Stahl, P. D., & Hodzic, D. (2006) · The Journal of Cell Biology · doi:10.1083/jcb.200509124 · Cited by 1,335
Self-citationLoss of a-Type Lamin Expression Compromises Nuclear Envelope Integrity Leading to Muscular Dystrophy
Sullivan, T., Escalante-Alcalde, D., Bhatt, H., Anver, M., Bhat, N., Nagashima, K., Stewart, C. L., & Burke, B. (1999) · The Journal of Cell Biology · doi:10.1083/jcb.147.5.913 · Cited by 1,211
Goldman, R. D., Shumaker, D. K., Erdos, M. R., Eriksson, M., Goldman, A. E., Gordon, L. B., Gruenbaum, Y., Khuon, S., Mendez, M., Varga, R., et al. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0402943101 · Cited by 1,103
Lamin A/C deficiency causes defective nuclear mechanics and mechanotransduction
Lammerding, J., Schulze, P. C., Takahashi, T., Kozlov, S. V., Sullivan, T. L., Kamm, R. D., Stewart, C. L., & Lee, R. T. (2004) · Journal of Clinical Investigation · doi:10.1172/jci19670 · Cited by 1,064
Identification of a novel X-linked gene responsible for Emery-Dreifuss muscular dystrophy
Bione, S., Maestrini, E., Rivella, S., Mancini, M., Regis, S., Romeo, G., & Toniolo, D. (1994) · Nature Genetics · doi:10.1038/ng1294-323 · Cited by 888
“Laminopathies”: A wide spectrum of human diseases
Worman, H. J., & Bonne, G. (2007) · Experimental Cell Research · doi:10.1016/j.yexcr.2007.03.028 · Cited by 661
Zhang, Q., Bethmann, C., Worth, N. F., Davies, J. D., Wasner, C., Feuer, A., Ragnauth, C. D., Yi, Q., Mellad, J. A., Warren, D. T., et al. (2007) · Human Molecular Genetics · doi:10.1093/hmg/ddm238 · Cited by 539
Self-citationHaque, F., Lloyd, D. J., Smallwood, D. T., Dent, C. L., Shanahan, C. M., Fry, A. M., Trembath, R. C., & Shackleton, S. (2006) · Molecular and Cellular Biology · doi:10.1128/mcb.26.10.3738-3751.2006 · Cited by 503
Self-citationWilhelmsen, K., Litjens, S. H. M., Kuikman, I., Tshimbalanga, N., Janssen, H., van den Bout, I., Raymond, K., & Sonnenberg, A. (2005) · The Journal of Cell Biology · doi:10.1083/jcb.200506083 · Cited by 483
Cited by
Koch, A. J., & Holaska, J. M. (2014) · Seminars in Cell & Developmental Biology · doi:10.1016/j.semcdb.2013.12.008 · Cited by 114
Nuclear Mechanotransduction in Skeletal Muscle
Jabre, S., Hleihel, W., & Coirault, C. (2021) · Cells · doi:10.3390/cells10020318 · Cited by 31
LINCing Nuclear Mechanobiology With Skeletal Muscle Mass and Function
van Ingen, M. J. A., & Kirby, T. J. (2021) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2021.690577 · Cited by 26
Metadata
- Authors
- Haque, F., Mazzeo, D., Patel, J. T., Smallwood, D. T., Ellis, J. A., Shanahan, C. M., & Shackleton, S.
- Venue
- Journal of Biological Chemistry
- Publisher
- Elsevier BV
- Published
- 2010-01
- Volume
- 285
- Issue
- 5
- Pages
- 3487-3498
- ISSN
- 0021-9258 (ISSN-L)
- OpenAlex
- W2076228191
- Cited by
- 287
- References
- 64
- FWCI
- 7.08
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Haque, F., Mazzeo, D., Patel, J. T., Smallwood, D. T., Ellis, J. A., Shanahan, C. M., & Shackleton, S. (2010). Mammalian SUN Protein Interaction Networks at the Inner Nuclear Membrane and Their Role in Laminopathy Disease Processes. Journal of Biological Chemistry. https://doi.org/10.1074/jbc.m109.071910
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