AI Article Synopsis
- * Mutations in the GAS2 gene, which affects cytoskeletal organization, lead to hearing loss by destabilizing microtubule bundles in supporting cells, resulting in reduced stiffness and altered cochlear mechanics.
- * Studies in mice showed that these GAS2 mutations caused defects in how sound waves travel and amplify within the cochlea, highlighting the crucial role of GAS2 in maintaining the mechanical properties of supporting cells for effective sound transmission.
Article Abstract
In mammals, sound is detected by mechanosensory hair cells that are activated in response to vibrations at frequency-dependent positions along the cochlear duct. We demonstrate that inner ear supporting cells provide a structural framework for transmitting sound energy through the cochlear partition. Humans and mice with mutations in GAS2, encoding a cytoskeletal regulatory protein, exhibit hearing loss due to disorganization and destabilization of microtubule bundles in pillar and Deiters' cells, two types of inner ear supporting cells with unique cytoskeletal specializations. Failure to maintain microtubule bundle integrity reduced supporting cell stiffness, which in turn altered cochlear micromechanics in Gas2 mutants. Vibratory responses to sound were measured in cochleae from live mice, revealing defects in the propagation and amplification of the traveling wave in Gas2 mutants. We propose that the microtubule bundling activity of GAS2 imparts supporting cells with mechanical properties for transmitting sound energy through the cochlea.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8137675 | PMC |
| http://dx.doi.org/10.1016/j.devcel.2021.04.017 | DOI Listing |
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