Radixin modulates the function of outer hair cell stereocilia

Autor: Guney Bademci, Asli Subasioglu, María Costales, Rocío González-Aguado, Ana Fontalba, Anders Fridberger, Sonal Prasad, Juan Cadiñanos, Mustafa Tekin, Marta Diñeiro, Barbara Vona, Rubén Cabanillas, Clara Diego-Pérez
Jazyk: angličtina
Rok vydání: 2020
Předmět:
0301 basic medicine
Stereocilia (inner ear)
Medicine (miscellaneous)
Fluorescent Antibody Technique
Gene Expression
Stimulation
Mechanotransduction
Cellular
Arsenicals
0302 clinical medicine
Radixin
Inner ear
Biology (General)
Chemistry
Molecular biophysics
Cell biology
Pedigree
medicine.anatomical_structure
Child
Preschool
Female
Hair cell
General Agricultural and Biological Sciences
Genotype
Cellbiologi
QH301-705.5
Guinea Pigs
Motility
Sensory system
macromolecular substances
Models
Biological
General Biochemistry
Genetics and Molecular Biology
Article
Stereocilia
03 medical and health sciences
medicine
otorhinolaryngologic diseases
Animals
Humans
Hearing Loss
Alleles
Genetic Variation
Membrane Proteins
Cell Biology
Cytoskeletal Proteins
Disease Models
Animal
Hair Cells
Auditory
Outer
030104 developmental biology
Acoustic Stimulation
sense organs
030217 neurology & neurosurgery
Function (biology)
Zdroj: Communications Biology
Communications Biology, Vol 3, Iss 1, Pp 1-15 (2020)
ISSN: 2399-3642
Popis: 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.
Sonal Prasad et al. identify several mutations in the radixin (RDX) gene that are associated with early-life hearing loss. Using a guinea pig model, they propose that radixin helps convert sound into electrical signals in the mature inner ear.
Databáze: OpenAIRE
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