Myosin-VIIa is expressed in multiple isoforms and essential for tensioning the hair cell mechanotransduction complex

Sihan Li, Andrew Mecca, Jeewoo Kim, Giusy A. Caprara, Elizabeth L. Wagner, Ting-Ting Du, Leonid Petrov, Wenhao Xu, Runjia Cui, Ivan T. Rebustini, Bechara Kachar, Anthony W. Peng () and Jung-Bum Shin ()
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Sihan Li: University of Virginia
Andrew Mecca: University of Colorado Anschutz Medical Campus
Jeewoo Kim: University of Virginia
Giusy A. Caprara: University of Colorado Anschutz Medical Campus
Elizabeth L. Wagner: University of Virginia
Ting-Ting Du: University of Virginia
Leonid Petrov: University of Virginia
Wenhao Xu: University of Virginia
Runjia Cui: National Institute of Health
Ivan T. Rebustini: National Institute of Health
Bechara Kachar: National Institute of Health
Anthony W. Peng: University of Colorado Anschutz Medical Campus
Jung-Bum Shin: University of Virginia

Nature Communications, 2020, vol. 11, issue 1, 1-15

Abstract: Abstract Mutations in myosin-VIIa (MYO7A) cause Usher syndrome type 1, characterized by combined deafness and blindness. MYO7A is proposed to function as a motor that tensions the hair cell mechanotransduction (MET) complex, but conclusive evidence is lacking. Here we report that multiple MYO7A isoforms are expressed in the mouse cochlea. In mice with a specific deletion of the canonical isoform (Myo7a-ΔC mouse), MYO7A is severely diminished in inner hair cells (IHCs), while expression in outer hair cells is affected tonotopically. IHCs of Myo7a-ΔC mice undergo normal development, but exhibit reduced resting open probability and slowed onset of MET currents, consistent with MYO7A’s proposed role in tensioning the tip link. Mature IHCs of Myo7a-ΔC mice degenerate over time, giving rise to progressive hearing loss. Taken together, our study reveals an unexpected isoform diversity of MYO7A expression in the cochlea and highlights MYO7A’s essential role in tensioning the hair cell MET complex.

Date: 2020
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DOI: 10.1038/s41467-020-15936-z

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