Journal
JOURNAL OF NEUROSCIENCE
Volume 30, Issue 29, Pages 9683-9694Publisher
SOC NEUROSCIENCE
DOI: 10.1523/JNEUROSCI.1541-10.2010
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Funding
- National Institutes of Health (NIH) [DC009412, R01 DC002368, R21 DC008801, P30 DC005983, DC005827]
- NIH National Cancer Institute [CA34196]
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The quantitative trait locus ahl8 is a key contributor to the early-onset, age-related hearing loss of DBA/2J mice. A nonsynonymous nucleotide substitution in the mouse fascin-2 gene (Fscn2) is responsible for this phenotype, confirmed by wild-type BAC transgene rescue of hearing loss in DBA/2J mice. In chickens and mice, FSCN2 protein is abundant in hair-cell stereocilia, the actin-rich structures comprising the mechanically sensitive hair bundle, and is concentrated toward stereocilia tips of the bundle's longest stereocilia. FSCN2 expression increases when these stereocilia differentially elongate, suggesting that FSCN2 controls filament growth, stiffens exposed stereocilia, or both. Because ahl8 accelerates hearing loss only in the presence of mutant cadherin 23, a component of hair-cell tip links, mechanotransduction and actin crosslinking must be functionally interrelated.
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