Journal
BIRTH DEFECTS RESEARCH PART C-EMBRYO TODAY-REVIEWS
Volume 105, Issue 2, Pages 126-139Publisher
WILEY
DOI: 10.1002/bdrc.21098
Keywords
hair cell; hearing; cuticular plate; mechanotransduction
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Funding
- NIDCD NIH HHS [R01 DC009437, DC009437] Funding Source: Medline
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The mechanosensitive hair cells of the inner ear are crucial to hearing and vestibular function. Each hair cell detects the mechanical stimuli associated with sound or head movement with a hair bundle at the apical surface of the cell, consisting of a precise array of actin-based stereocilia. Each stereocilium inserts as a rootlet into a dense filamentous actin mesh known as the cuticular plate. Disruption of the parallel actin bundles forming the stereocilia results in hearing impairments and balance defects. The cuticular plate is thought to be involved in holding the stereocilia in place. However, the precise role of the cuticular plate in hair bundle development, maintenance, and hearing remains unknown. Ultrastructural studies have revealed a complex cytoskeletal architecture, but a lack of knowledge of proteins that inhabit the cuticular plate and a dearth of mutations that perturb relevant proteins have hindered our understanding of the functions of the cuticular plate. Here, we discuss what is known about the structure and development of this unique and poorly-understood actin-rich organelle. Birth Defects Research (Part C) 105:126-139, 2015. (c) 2015 Wiley Periodicals, Inc.
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