Radixin modulates the function of outer hair cell stereocilia

Commun Biol. 2020 Dec 23;3(1):792. doi: 10.1038/s42003-020-01506-y.

Abstract

The stereocilia of the inner ear sensory cells contain the actin-binding protein radixin, encoded by RDX. Radixin is important for hearing but remains functionally obscure. To determine how radixin influences hearing sensitivity, we used a custom rapid imaging technique to visualize stereocilia motion while measuring electrical potential amplitudes during acoustic stimulation. Radixin inhibition decreased sound-evoked electrical potentials. Other functional measures, including electrically induced sensory cell motility and sound-evoked stereocilia deflections, showed a minor amplitude increase. These unique functional alterations demonstrate radixin as necessary for conversion of sound into electrical signals at acoustic rates. We identified patients with RDX variants with normal hearing at birth who showed rapidly deteriorating hearing during the first months of life. This may be overlooked by newborn hearing screening and explained by multiple disturbances in postnatal sensory cells. We conclude radixin is necessary for ensuring normal conversion of sound to electrical signals in the inner ear.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acoustic Stimulation
  • Alleles
  • Animals
  • Arsenicals / pharmacology
  • Child, Preschool
  • Cytoskeletal Proteins / genetics
  • Cytoskeletal Proteins / metabolism*
  • Disease Models, Animal
  • Female
  • Fluorescent Antibody Technique
  • Gene Expression
  • Genetic Variation
  • Genotype
  • Guinea Pigs
  • Hair Cells, Auditory, Outer / drug effects
  • Hair Cells, Auditory, Outer / metabolism*
  • Hearing Loss / diagnosis
  • Hearing Loss / genetics
  • Humans
  • Mechanotransduction, Cellular / genetics
  • Membrane Proteins / genetics
  • Membrane Proteins / metabolism*
  • Models, Biological
  • Pedigree
  • Stereocilia / drug effects
  • Stereocilia / metabolism*

Substances

  • Arsenicals
  • Cytoskeletal Proteins
  • Membrane Proteins
  • radixin
  • benzenearsonic acid