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

Author

Listed:
  • Sihan Li

    (University of Virginia
    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
    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
    University of Virginia)

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.

Suggested Citation

  • 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 & Jung-Bum , 2020. "Myosin-VIIa is expressed in multiple isoforms and essential for tensioning the hair cell mechanotransduction complex," Nature Communications, Nature, vol. 11(1), pages 1-15, December.
    • Handle: RePEc:nat:natcom:v:11:y:2020:i:1:d:10.1038_s41467-020-15936-z
    DOI: 10.1038/s41467-020-15936-z
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