Radixin modulates the function of outer hair cell stereocilia.
Acoustic Stimulation
Alleles
Animals
Arsenicals
/ pharmacology
Child, Preschool
Cytoskeletal Proteins
/ genetics
Disease Models, Animal
Female
Fluorescent Antibody Technique
Gene Expression
Genetic Variation
Genotype
Guinea Pigs
Hair Cells, Auditory, Outer
/ drug effects
Hearing Loss
/ diagnosis
Humans
Mechanotransduction, Cellular
/ genetics
Membrane Proteins
/ genetics
Models, Biological
Pedigree
Stereocilia
/ drug effects
Journal
Communications biology
ISSN: 2399-3642
Titre abrégé: Commun Biol
Pays: England
ID NLM: 101719179
Informations de publication
Date de publication:
23 12 2020
23 12 2020
Historique:
received:
04
03
2020
accepted:
18
11
2020
entrez:
28
12
2020
pubmed:
29
12
2020
medline:
22
6
2021
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 33361775
doi: 10.1038/s42003-020-01506-y
pii: 10.1038/s42003-020-01506-y
pmc: PMC7758333
doi:
Substances chimiques
Arsenicals
0
Cytoskeletal Proteins
0
Membrane Proteins
0
radixin
144517-21-5
benzenearsonic acid
57F9KU116M
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
792Subventions
Organisme : NIDCD NIH HHS
ID : R01 DC009645
Pays : United States
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