Sensing sound: Cellular specializations and molecular force sensors

X Qiu, U Müller - Neuron, 2022 - cell.com
Organisms of all phyla express mechanosensitive ion channels with a wide range of
physiological functions. In recent years, several classes of mechanically gated ion channels …

Beyond cell–cell adhesion: sensational cadherins for hearing and balance

A Jaiganesh, Y Narui… - Cold Spring …, 2018 - cshperspectives.cshlp.org
Cadherins form a large family of proteins often involved in calcium-dependent cellular
adhesion. Although classical members of the family can provide a physical bond between …

Zooming in on cadherin-23: structural diversity and potential mechanisms of inherited deafness

A Jaiganesh, P De-la-Torre, AA Patel, DJ Termine… - Structure, 2018 - cell.com
Summary Cadherin-23 (CDH23) is an essential component of hair-cell tip links, fine
filaments that mediate inner-ear mechanotransduction. The extracellular domain of CDH23 …

Myosin 7 and its adaptors link cadherins to actin

IM Yu, VJ Planelles-Herrero, Y Sourigues… - Nature …, 2017 - nature.com
Cadherin linkages between adjacent stereocilia and microvilli are essential for
mechanotransduction and maintaining their organization. They are anchored to actin …

Myosin VII, USH1C, and ANKS4B or USH1G together form condensed molecular assembly via liquid-liquid phase separation

Y He, J Li, M Zhang - Cell reports, 2019 - cell.com
Hair cell stereocilia tip-links function to sense mechanical forces generated by sound waves
and maintain the structure of stereocilia by rooting the tail of cadherins to highly dense …

RBM24 is required for mouse hair cell development through regulating pre‐mRNA alternative splicing and mRNA stability

Y Wang, C Zhang, W Peng, H Du… - Journal of Cellular …, 2023 - Wiley Online Library
As the sensory receptor cells in vertebrate inner ear and lateral lines, hair cells are
characterized by the hair bundle that consists of one tubulin‐based kinocilium and dozens of …

Keratan sulfate, an electrosensory neurosentient bioresponsive cell instructive glycosaminoglycan

J Melrose - Glycobiology, 2024 - academic.oup.com
The roles of keratan sulfate (KS) as a proton detection glycosaminoglycan in neurosensory
processes in the central and peripheral nervous systems is reviewed. The functional …

Whirlin and PDZ domain-containing 7 (PDZD7) proteins are both required to form the quaternary protein complex associated with Usher syndrome type 2

Q Chen, J Zou, Z Shen, W Zhang, J Yang - Journal of Biological Chemistry, 2014 - ASBMB
Usher syndrome (USH) is the leading genetic cause of combined hearing and vision loss.
Among the three USH clinical types, type 2 (USH2) occurs most commonly. USH2A, GPR98 …

Disruption of Cdh23 exon 68 splicing leads to progressive hearing loss in mice by affecting tip-link stability

N Li, S Liu, D Zhao, H Du, Y **, X Wei… - Proceedings of the …, 2024 - National Acad Sciences
Inner ear hair cells are characterized by the F-actin-based stereocilia that are arranged into
a staircase-like pattern on the apical surface of each hair cell. The tips of shorter-row …

Zebrafish models of human eye and inner ear diseases

B Blanco-Sánchez, A Clément, JB Phillips… - Methods in cell …, 2017 - Elsevier
Eye and inner ear diseases are the most common sensory impairments that greatly impact
quality of life. Zebrafish have been intensively employed to understand the fundamental …