期刊
NATURE COMMUNICATIONS
卷 10, 期 -, 页码 -出版社
NATURE RESEARCH
DOI: 10.1038/s41467-019-11712-w
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资金
- National Institute of Neurological Disorders and Stroke [P30-NS072030]
- NCI Cancer Center Support Grant [P30-CA006516]
- National Institute on Deafness and Other Communication Disorders [R01-DC002881, R01-DC016932, R01-DC017166, R01DC017166-S1]
- National Science Foundation under NSF [1541959]
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.
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