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

Xudong Wu, Maryna V. Ivanchenko, Hoor Al Jandal, Marcelo Cicconet, Artur A. Indzhykulian () and David P. Corey ()
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Xudong Wu: Harvard Medical School
Maryna V. Ivanchenko: Harvard Medical School
Hoor Al Jandal: Harvard Medical School
Marcelo Cicconet: Image and Data Analysis Core, Harvard Medical School
Artur A. Indzhykulian: Harvard Medical School
David P. Corey: Harvard Medical School

Nature Communications, 2019, vol. 10, issue 1, 1-15

Abstract: 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.

Date: 2019
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DOI: 10.1038/s41467-019-11712-w

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