title: Stereocilia morphogenesis and maintenance through regulation of actin stability.
abstract: Stereocilia are actin-based protrusions on auditory and vestibular sensory cells that are required for hearing and balance. They convert physical force from sound, head movement or gravity into an electrical signal, a process that is called mechanoelectrical transduction. This function depends on the ability of sensory cells to grow stereocilia of defined lengths. These protrusions form a bundle with a highly precise geometry that is required to detect nanoscale movements encountered in the inner ear. Congenital or progressive stereocilia degeneration causes hearing loss. Thus, understanding stereocilia hair bundle structure, development, and maintenance is pivotal to understanding the pathogenesis of deafness. Stereocilia cores are made from a tightly packed array of parallel, crosslinked actin filaments, the length and stability of which are regulated in part by myosin motors, actin crosslinkers and capping proteins. This review aims to describe stereocilia actin regulation in the context of an emerging "tip turnover" model where actin assembles and disassembles at stereocilia tips while the remainder of the core is exceptionally stable.
authors: Department of Biology, Indiana University-Purdue University Indianapolis, Indianapolis, IN 46022, USA.
authors: Jamis
authors: J
authors: McGrath
authors: Department of Biology, Indiana University-Purdue University Indianapolis, Indianapolis, IN 46022, USA.
authors: Pallabi
authors: P
authors: Roy
authors: Department of Biology, Indiana University-Purdue University Indianapolis, Indianapolis, IN 46022, USA. Electronic address: bperrin@iupui.edu.
authors: Benjamin J
authors: BJ
authors: Perrin
mesh_terms: D008841:Actin Cytoskeleton / Q000378:metabolism / Q000648:ultrastructure; D000199:Actins / Q000235:genetics* / Q000378:metabolism; D000818:Animals; D000070585:Cadherin Related Proteins; D015820:Cadherins / Q000235:genetics / Q000378:metabolism; D003638:Deafness / Q000473:pathology / Q000503:physiopathology; D015870:Gene Expression; D006198:Hair Cells, Auditory / Q000378:metabolism / Q000648:ultrastructure*; D018069:Hair Cells, Vestibular / Q000378:metabolism / Q000648:ultrastructure*; D006309:Hearing / Q000502:physiology*; D040542:Mechanotransduction, Cellular; D051379:Mice; D008954:Models, Biological; D009024:Morphogenesis; D009218:Myosins / Q000235:genetics / Q000378:metabolism; D011498:Protein Precursors / Q000235:genetics / Q000378:metabolism; D055550:Protein Stability; D059547:Stereocilia / Q000378:metabolism / Q000648:ultrastructure*
chemical_list: D000199:Actins; C418976:CDHR15 protein, mouse; D000070585:Cadherin Related Proteins; D015820:Cadherins; C499091:Cdh23 protein, mouse; D011498:Protein Precursors; D009218:Myosins
keywords: Actin; Deafness; Hearing; Stereocilia