Human deafness-associated variants alter the dynamics of key molecules in hair cell stereocilia F-actin cores.

ABSTRACT: Stereocilia protrude up to 100 µm from the apical surface of vertebrate inner ear hair cells and are packed with cross-linked filamentous actin (F-actin). They function as mechanical switches to convert sound vibration into electrochemical neuronal signals transmitted to the brain. Several genes encode molecular components of stereocilia including actin monomers, actin regulatory and bundling proteins, motor proteins and the proteins of the mechanotransduction complex. A stereocilium F-actin core is a dynamic system, which is continuously being remodeled while maintaining an outwardly stable architecture under the regulation of F-actin barbed-end cappers, severing proteins and crosslinkers. The F-actin cores of stereocilia also provide a pathway for motor proteins to transport cargos inclu

SUBMITTER: Miyoshi T 

PROVIDER: S-EPMC11351816 | biostudies-literature | 2022 Apr

REPOSITORIES: biostudies-literature