TRIOBP-5 sculpts stereocilia rootlets and stiffens supporting cells enabling hearing
نویسندگان
چکیده
منابع مشابه
Actin-Bundling Protein TRIOBP Forms Resilient Rootlets of Hair Cell Stereocilia Essential for Hearing
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 p...
متن کاملWrapping up Stereocilia Rootlets
The actin-filled stereocilia of cochlear hair cells deflect in response to sound by pivoting around rootlets at their insertion points at the sensory cell surface. Kitajiri et al. (2010) now show that the actin-binding protein TRIOBP tightly bundles the actin filaments into rootlets, endowing these stereocilia "pivots" with unique elasticity and robustness.
متن کاملA novel splice site mutation of myosin VI in mice leads to stereociliary fusion caused by disruption of actin networks in the apical region of inner ear hair cells
An unconventional myosin encoded by the myosin VI gene (MYO6) contributes to hearing loss in humans. Homozygous mutations of MYO6 result in nonsyndromic profound congenital hearing loss, DFNB37. Kumamoto shaker/waltzer (ksv) mice harbor spontaneous mutations, and homozygous mutants exhibit congenital defects in balance and hearing caused by fusion of the stereocilia. We identified a Myo6c.1381G...
متن کاملSupporting cells contribute to control of hearing sensitivity.
The mammalian hearing organ, the organ of Corti, was studied in an in vitro preparation of the guinea pig temporal bone. As in vivo, the hearing organ responded with an electrical potential, the cochlear microphonic potential, when stimulated with a test tone. After exposure to intense sound, the response to the test tone was reduced. The electrical response either recovered within 10-20 min or...
متن کاملFate of mammalian cochlear hair cells and stereocilia after loss of the stereocilia.
Cochlear hair cells transduce mechanical stimuli into electrical activity. The site of hair cell transduction is the hair bundle, an array of stereocilia with different height arranged in a staircase. Tip links connect the apex of each stereocilium to the side of its taller neighbor. The hair bundle and tip links of hair cells are susceptible to acoustic trauma and ototoxic drugs. It has been s...
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ژورنال
عنوان ژورنال: JCI Insight
سال: 2019
ISSN: 2379-3708
DOI: 10.1172/jci.insight.128561