Actin-Bundling Protein TRIOBP Forms Resilient Rootlets of Hair Cell Stereocilia Essential for Hearing
In: Cell, Jg. 141 (2010-05-01), S. 786-798
Online
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Zugriff:
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 crosslinker in stereocilia. We generated mutant Triobp mice (Triobp(Deltaex8/Deltaex8)) that are profoundly deaf. Stereocilia of Triobp(Deltaex8/Deltaex8) 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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Actin-Bundling Protein TRIOBP Forms Resilient Rootlets of Hair Cell Stereocilia Essential for Hearing
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Autor/in / Beteiligte Person: | Griffith, Andrew J. ; Hammer, John A. ; Stepanyan, Ruben ; Frolenkov, Gregory I. ; Friedman, Thomas B. ; Richardson, Guy P. ; Hinshaw, Jenny E. ; Fujiwara, Ikuko ; Kitajiri, Shin-ichiro ; Bartles, James R. ; Ahmed, Zubair M. ; Riazuddin, Sheikh ; Sellers, James R. ; Bird, Jonathan E. ; Goodyear, Richard J. ; Riazuddin, Saima ; Belyantseva, Inna A. ; Sakamoto, Takeshi |
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Zeitschrift: | Cell, Jg. 141 (2010-05-01), S. 786-798 |
Veröffentlichung: | Elsevier BV, 2010 |
Medientyp: | unknown |
ISSN: | 0092-8674 (print) |
DOI: | 10.1016/j.cell.2010.03.049 |
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