Journal
CELL
Volume 141, Issue 5, Pages 786-798Publisher
CELL PRESS
DOI: 10.1016/j.cell.2010.03.049
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Funding
- NIDCD/NIH [R01DC008861, R01DC009434, R01 DC004314]
- Wellcome Trust [071394/Z/03/Z]
- HEC
- MoST, Islamabad
- NIDDK
- NHLBI
- NIDCD [Z01 DK060100, Z01 HL004232-08, Z01 DC 000064, Z01 DC000048]
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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(Delta ex8/Delta ex8)) that are profoundly deaf. Stereocilia of Triobp(Delta ex8/Delta ex8) 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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