Stereocilia morphogenesis and maintenance through regulation of actin stability

Semin Cell Dev Biol. 2017 May:65:88-95. doi: 10.1016/j.semcdb.2016.08.017. Epub 2016 Aug 23.

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.

Keywords: Actin; Deafness; Hearing; Stereocilia.

Publication types

  • Review

MeSH terms

  • Actin Cytoskeleton / metabolism
  • Actin Cytoskeleton / ultrastructure
  • Actins / genetics*
  • Actins / metabolism
  • Animals
  • Cadherin Related Proteins
  • Cadherins / genetics
  • Cadherins / metabolism
  • Deafness / pathology
  • Deafness / physiopathology
  • Gene Expression
  • Hair Cells, Auditory / metabolism
  • Hair Cells, Auditory / ultrastructure*
  • Hair Cells, Vestibular / metabolism
  • Hair Cells, Vestibular / ultrastructure*
  • Hearing / physiology*
  • Mechanotransduction, Cellular*
  • Mice
  • Models, Biological
  • Morphogenesis
  • Myosins / genetics
  • Myosins / metabolism
  • Protein Precursors / genetics
  • Protein Precursors / metabolism
  • Protein Stability
  • Stereocilia / metabolism
  • Stereocilia / ultrastructure*

Substances

  • Actins
  • CDHR15 protein, mouse
  • Cadherin Related Proteins
  • Cadherins
  • Cdh23 protein, mouse
  • Protein Precursors
  • Myosins