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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.
There is little known about the function or molecular identity of the electron-dense stereocilia coat, which is transiently present at the surface of stereocilia. In this study authors screened a database of hair-cell-enriched translated proteins to identify the expression of Polycystic Kidney and Hepatic Disease 1-Like 1 (PKHD1L1), a large, mostly extracellular protein, and show that it forms the coat at the tips of stereocilia and is required for normal hearing in mice
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1 Harvard Medical School, Department of Neurobiology, Boston, USA (GRID:grid.38142.3c) (ISNI:000000041936754X); Decibel Therapeutics, Boston, USA (GRID:grid.38142.3c)
2 Harvard Medical School, Department of Neurobiology, Boston, USA (GRID:grid.38142.3c) (ISNI:000000041936754X)
3 Harvard Medical School, Department of Neurobiology, Boston, USA (GRID:grid.38142.3c) (ISNI:000000041936754X); Northeastern University, Boston, USA (GRID:grid.261112.7) (ISNI:0000 0001 2173 3359)
4 Image and Data Analysis Core, Harvard Medical School, Boston, USA (GRID:grid.38142.3c) (ISNI:000000041936754X)
5 Harvard Medical School, Department of Neurobiology, Boston, USA (GRID:grid.38142.3c) (ISNI:000000041936754X); Harvard Medical School and Massachusetts Eye and Ear, Department of Otolaryngology, Boston, USA (GRID:grid.39479.30) (ISNI:0000 0000 8800 3003)