Usher proteins in inner ear structure and function.

  title={Usher proteins in inner ear structure and function.},
  author={Z M Ahmed and Gregory I Frolenkov and Saima Riazuddin},
  journal={Physiological genomics},
  volume={45 21},
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… CONTINUE READING

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