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Physiol Genomics. 2013 Nov 1;45(21):987-9. doi: 10.1152/physiolgenomics.00135.2013. Epub 2013 Sep 10.

Usher proteins in inner ear structure and function.

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  • 1Division of Pediatric Ophthalmology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Ohio;


Usher syndrome (USH) is a neurosensory disorder affecting both hearing and vision in humans. Linkage studies of families of USH patients, studies in animals, and characterization of purified proteins have provided insight into the molecular mechanisms of hearing. To date, 11 USH proteins have been identified, and evidence suggests that all of them are crucial for the function of the mechanosensory cells of the inner ear, the hair cells. Most USH proteins are localized to the stereocilia of the hair cells, where mechano-electrical transduction (MET) of sound-induced vibrations occurs. Therefore, elucidation of the functions of USH proteins in the stereocilia is a prerequisite to understanding the exact mechanisms of MET.


CIB2; Usher syndrome; calcium buffer; inner ear; retinitis pigmentosa

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