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PKHD1L1 is a coat protein of hair-cell stereocilia and is required for normal hearing

Author

Listed:
  • Xudong Wu

    (Harvard Medical School
    Decibel Therapeutics)

  • Maryna V. Ivanchenko

    (Harvard Medical School)

  • Hoor Al Jandal

    (Harvard Medical School
    Northeastern University)

  • Marcelo Cicconet

    (Image and Data Analysis Core, Harvard Medical School)

  • Artur A. Indzhykulian

    (Harvard Medical School
    Harvard Medical School and Massachusetts Eye and Ear)

  • David P. Corey

    (Harvard Medical School)

Abstract

The bundle of stereocilia on inner ear hair cells responds to subnanometer deflections produced by sound or head movement. Stereocilia are interconnected by a variety of links and also carry an electron-dense surface coat. The coat may contribute to stereocilia adhesion or protect from stereocilia fusion, but its molecular identity remains unknown. From a database of hair-cell-enriched translated proteins, we identify Polycystic Kidney and Hepatic Disease 1-Like 1 (PKHD1L1), a large, mostly extracellular protein of 4249 amino acids with a single transmembrane domain. Using serial immunogold scanning electron microscopy, we show that PKHD1L1 is expressed at the tips of stereocilia, especially in the high-frequency regions of the cochlea. PKHD1L1-deficient mice lack the surface coat at the upper but not lower regions of stereocilia, and they develop progressive hearing loss. We conclude that PKHD1L1 is a component of the surface coat and is required for normal hearing in mice.

Suggested Citation

  • Xudong Wu & Maryna V. Ivanchenko & Hoor Al Jandal & Marcelo Cicconet & Artur A. Indzhykulian & David P. Corey, 2019. "PKHD1L1 is a coat protein of hair-cell stereocilia and is required for normal hearing," Nature Communications, Nature, vol. 10(1), pages 1-15, December.
    • Handle: RePEc:nat:natcom:v:10:y:2019:i:1:d:10.1038_s41467-019-11712-w
    DOI: 10.1038/s41467-019-11712-w
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    Cited by:

    1. Katharine K. Miller & Pei Wang & Nicolas Grillet, 2024. "SUB-immunogold-SEM reveals nanoscale distribution of submembranous epitopes," Nature Communications, Nature, vol. 15(1), pages 1-13, December.
    2. Maryna V. Ivanchenko & Daniel M. Hathaway & Alex J. Klein & Bifeng Pan & Olga Strelkova & Pedro De-la-Torre & Xudong Wu & Cole W. Peters & Eric M. Mulhall & Kevin T. Booth & Corey Goldstein & Joseph B, 2023. "Mini-PCDH15 gene therapy rescues hearing in a mouse model of Usher syndrome type 1F," Nature Communications, Nature, vol. 14(1), pages 1-21, December.
    3. Pei Wang & Katharine K. Miller & Enqi He & Siddhant S. Dhawan & Christopher L. Cunningham & Nicolas Grillet, 2024. "LOXHD1 is indispensable for maintaining TMC1 auditory mechanosensitive channels at the site of force transmission," Nature Communications, Nature, vol. 15(1), pages 1-21, December.

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