Grxcr2 is required for stereocilia morphogenesis in the cochlea

PloS One
Matthew R AvenariusD C Kohrman

Abstract

Hearing and balance depend upon the precise morphogenesis and mechanosensory function of stereocilia, the specialized structures on the apical surface of sensory hair cells in the inner ear. Previous studies of Grxcr1 mutant mice indicated a critical role for this gene in control of stereocilia dimensions during development. In this study, we analyzed expression of the paralog Grxcr2 in the mouse and evaluated auditory and vestibular function of strains carrying targeted mutations of the gene. Peak expression of Grxcr2 occurs during early postnatal development of the inner ear and GRXCR2 is localized to stereocilia in both the cochlea and in vestibular organs. Homozygous Grxcr2 deletion mutants exhibit significant hearing loss by 3 weeks of age that is associated with developmental defects in stereocilia bundle orientation and organization. Despite these bundle defects, the mechanotransduction apparatus assembles in relatively normal fashion as determined by whole cell electrophysiological evaluation and FM1-43 uptake. Although Grxcr2 mutants do not exhibit overt vestibular dysfunction, evaluation of vestibular evoked potentials revealed subtle defects of the mutants in response to linear accelerations. In addition, reduced Grx...Continue Reading

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Citations

Feb 3, 2021·Molecular Genetics & Genomic Medicine·Ambroise WonkamGuillaume Pare
May 25, 2021·Frontiers in Cell and Developmental Biology·Jinan LiBo Zhao
Aug 10, 2021·Frontiers in Cellular Neuroscience·Chang Liu, Bo Zhao

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Methods Mentioned

BETA
targeted mutation
NMR
PCR
biopsies
chip
Exome Sequencing
genotyping
scanning electron microscopy
RNA-Seq

Software Mentioned

Constraint Multiple Tool
COBALT
SIFT
MutationTaster
Psi
PyMOL Molecular Graphics System
dbNSFP
Ensembl
ImageJ
TASSER

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