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Actin-Bundling Protein TRIOBP Forms Resilient Rootlets of Hair Cell Stereocilia Essential for Hearing
Open accessAbstract
SUMMARY Inner ear hair cells detect sound through deflection of mechanosensory stereocilia. Each stereocilium is supported by a paracrystalline array of parallel actin filaments that are packed more densely at the base, forming a rootlet extending into the cell body. The function of rootlets and the molecules responsible for their formation are unknown. We found that TRIOBP, a cytoskeleton-associated protein mutated in human hereditary deafness DFNB28, is localized to rootlets. In vitro, purified TRIOBP isoform 4 protein organizes actin filaments into uniquely dense bundles reminiscent of rootlets, but distinct from bundles formed by espin, an actin cross-linker in stereocilia. We generated mutant Triobp mice (TriobpΔex8/Δex8) that are profoundly deaf. Stereocilia of TriobpΔex8/Δex8 mice develop normally, but fail to form rootlets and are easier to deflect and damage. Thus, F-actin bundling by TRIOBP provides durability and rigidity for normal mechanosensitivity of stereocilia and may contribute to resilient cytoskeletal structures elsewhere.
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Citations
Cites
Microscopic analysis of polymerization dynamics with individual actin filaments
Fujiwara, I., Takahashi, S., Tadakuma, H., Funatsu, T., & Ishiwata, S. (2002) · Nature Cell Biology · doi:10.1038/ncb841 · Cited by 420
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Cited by
Bowl, M. R., & Dawson, S. J. (2019) · Cold Spring Harbor Perspectives in Medicine · doi:10.1101/cshperspect.a033217 · Cited by 396
The Physiology of Mechanoelectrical Transduction Channels in Hearing
Fettiplace, R., & Kim, K. X. (2014) · Physiological Reviews · doi:10.1152/physrev.00038.2013 · Cited by 313
Higdon, C. W., Mitra, R. D., & Johnson, S. L. (2013) · PLoS ONE · doi:10.1371/journal.pone.0067801 · Cited by 125
Sp1 Suppresses miR-3178 to Promote the Metastasis Invasion Cascade via Upregulation of TRIOBP
Wang, H., Li, K., Mei, Y., Huang, X., Li, Z., Yang, Q., & Yang, H. (2018) · Molecular Therapy Nucleic Acids · doi:10.1016/j.omtn.2018.04.008 · Cited by 23
Metadata
- Authors
- Kitajiri, S.-I., Sakamoto, T., Belyantseva, I. A., Goodyear, R. J., Stepanyan, R., Fujiwara, I., Bird, J. E., Riazuddin, S., Riazuddin, S., Ahmed, Z. M., Hinshaw, J. E., Sellers, J., Bartles, J. R., Hammer, J. A., Richardson, G. P., Griffith, A. J., Frolenkov, G. I., & Friedman, T. B.
- Venue
- Cell
- Publisher
- Elsevier BV
- Published
- 2010-05
- Volume
- 141
- Issue
- 5
- Pages
- 786-798
- ISSN
- 0092-8674 (ISSN-L)
- OpenAlex
- W2084929485
- Cited by
- 201
- References
- 1
- FWCI
- 6.78
- Type
- journal article
- Language
- en
- Open access
- bronze
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- The paper
- Kitajiri, S.-I., Sakamoto, T., Belyantseva, I. A., Goodyear, R. J., Stepanyan, R., Fujiwara, I., Bird, J. E., Riazuddin, S., Riazuddin, S., Ahmed, Z. M., Hinshaw, J. E., Sellers, J., Bartles, J. R., Hammer, J. A., Richardson, G. P., Griffith, A. J., Frolenkov, G. I., & Friedman, T. B. (2010). Actin-Bundling Protein TRIOBP Forms Resilient Rootlets of Hair Cell Stereocilia Essential for Hearing. Cell. https://doi.org/10.1016/j.cell.2010.03.049
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